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The TOF Child This book is dedicated to TOF children and their families, in memory of Benjamin Pouncey.

About this book: Co-ordinated and edited by Vicki Martin BSc BVSc, with assistance from Sue Goodley (Nottingham Neonatal Service) and TOF parent Janita Clamp MA. Illustration & design/layout by Vicki Martin, Andrea Slane and Jeremy Dix of Vicki Martin Design (www.vetdesign.co.uk). Published by TOFS, St George's Centre, 91 Victoria Road, Netherfield, Nottingham NG4 2NN. Tel: 0115 961 3092 Fax: 0115 961 3097 Email: info@tofs.org.uk Web site: www.tofs.org.uk First published in 1999 ISBN 0 9536265 0 4

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The TOF Child

Contents Introduction .....................................4 Editorial ...........................................6 What is TOF / OA? ............................7 What is Wrong in TOF/OA? ...........8 The Need for Surgery .................. 10 Diagnosis ................................... 10 Antenatal Diagnosis of TOF/OA using Ultrasound ........................ 11 Conditions Occurring with TOF/OA ............................... 14 Transfer to Another Hospital ...... 14 On Learning Your Child is a TOF ....15 Father ......................................... Mother ........................................ Coming to Terms with Reality .... Post-Operative Period.................. Prenatal Diagnosis .....................

16 16 17 17 18

The Management of TOF/OA ...........21 Specialist Information: Transfer of the TOF/OA baby ……21 Pre-Operative Management ..........21 Long-gap Oesophageal Atresia ....24 Oesophageal Substitution Procedures ..................26 Colon Interposition .................... 27 Gastric Tube Oesophagoplasty .. 28 Gastric Transposition ................. 28 Post-Operative Care ..................... 29 Outlook ...................................... 29 The Post-Operative Care of Children with TOF/OA ................30 Monitoring ................................. 30 Infusions or Drips ...................... 31 Ventilators .................................. 32 Chest Drains ............................... 33 The Chest Wound ........................ 33 Feeding ....................................... 33 Recovery ......................................34 Other Problems ............................ 34

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Discharge from Hospital ................ Preparing for Discharge ............ Equipment ............................... Feeding.................................... Care of Nasogastric Tubes ........ Care of Gastrostomy Tubes ...... Gastro-Oesophageal Reflux ....... Tracheomalacia........................ Medicines ................................ Chest Infections ...................... Physiotherapy .......................... Additional Problems ................. Follow Up ................................ Genetic Counselling for Tracheo-Oesophageal Fistula ... What is it? ............................... Why did it Happen? .................. Will it Happen Again? ............... Can Anything be Done to Prevent a Recurrence? .........

37 38 39 39 42 43 44 45 45 47 47 47 48 49 49 50 50 51

Feeding the TOF Child .................. Breast Feeding ......................... Weaning and Solid Foods ......... Practical Tips on Feeding ......... Reluctance to Feed ................... Feed-Related Problems ............. Troublesome Foods .................. Feeding Difficulties .................. Summary................................

52 52 52 53 54 54 55 55 57

Strictures and Reflux .................... What is a Stricture? ................. Symptoms of Stricture ............ Diagnosis of Stricture .............. Treatment of Stricture .............. Complications of Stricture Dilatation ................. What is Reflux? ....................... Symptoms of Reflux ................. Diagnosis of Reflux .................. Treatment of Reflux .................

61 61 61 62 63 64 64 65 66 67


Respiratory Problems in TOFs ........ 71 The Respiratory System ............... 71 Tracheomalacia ...................................73 Infections ..............................................77 Outlook in the Longer Term ........ 80 Growth in TOF Children ................ 82 Introduction ..........................................82 Normal Growth ............................. 82 Factors Affecting Growth in Children ..........................................83 Growth of TOF Children....................83 Who to talk to if you are worried about your child's growth .......... 85 The TOF Family .............................. 86 Initial Anxieties ....................................86 Getting Help and Support ...............86 Spreading the Load ....................... 87 What to Tell the Child? ......................88 The Child's Viewpoint.........................89 Siblings .....................................................89 Play Therapy................................. 90 Friends ........................................................ 91 Growing up .................................... 92 Moving on .................................................93 Summary ....................................... 94 Long Term Outcome; Results of a Follow-up Study ......... 95 Feeding .............................................95 Chest Problems ...............................95 Exercise, Education and Lifestyle ............................................95 Height and Weight ........................ 96 Lung Function........................................ 96 Summary ....................................... 96

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VACTERL ......................................... 98 Diagnosis ................................... 98 Cause ........................................... 98 Antenatal Diagnosis .................... 99 Symptoms and Treatment ............ 99 The VACTERL Child .................. 103 Radial Deformities in VACTERL .. 106 Nomenclature............................ 106 The Forearm Anomaly: Radial Club Hand. .................... 106 Treatment of the Foreall n ........ 107 The Hand Anomaly ................... 111 Treatment of the Hand .............. 112 Late Reconstruction and Secondary Surgery ............ 115 Summary .................................. 115 The Role of the Professional; Who to Ask for Help ....................... 116 Parents' Worries ............................ 120 X-rays ....................................... 120 Antibiotics and Anaesthetics .... 120 Paediatric Resuscitation ................ 121 Glossary ........................................ 124 Index ............................................. 127


The TOF Child

Introduction by the TOFS group. When the parents of a baby diagnosed with tracheo-oesophageal fistula (TOF) or oesophageal atresia (OA) are first told of the problems which exist and the operation(s) necessary to correct any defects, they are not usually in a suitable frame of mind to absorb all the information given to them, let alone understand the technical language. As the news sinks in, questions arise; some can be difficult to ask and some will inevitably crop up at a time when there is no one to respond. It is hoped that many of these questions will be answered in this book. Later on, when their child leaves hospital, parents are often told that there should be no subsequent problems. This can make them reluctant to go back to the hospital with queries; however because the condition is comparatively rare, few General Practitioners have experience of tracheo-oesophageal fistula (TOF) or oesophageal atresia (OA). The lack of a local source of advice can be frustrating — or even frightening — for those involved. This book contains contributions from medical experts as well as from parents and TOFs themselves. The hopes are twofold; firstly that parents will benefit from reading the book, but also that medical professionals and nursing staff — many of whom may not have encountered many TOF children, if any at all — will gain insight into the problems which can occur in both the medical management and the day-to-day care of a child with TOF / OA or VACTERL (a group of conditions which can include TOF/OA).

One of the questions which parents may want to, but cannot or do not ask is "what are the chances of my child's survival?" The answer will naturally depend on the number, type and severity of the defects, but it is a fact that nowadays virtually no child dies from either tracheo-oesophageal fistula or oesophageal atresia. There is always a small risk involved in having an anaesthetic, and that would apply for any child, TOF or not. There may also be other problems of a more serious nature, such as a severe heart defect, but a child with just TOF and/or OA has excellent chances of survival. And how well they can do! Vicki Martin, who produced this book, was

Above: Richard Briers, patron of the TOFS group.

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The TOF Child born with TOF and OA. Having qualified as a veterinary surgeon, she decided to combine her knowledge with her artistic talents to follow a career as a medical illustrator and designer. She is a keen sportswoman, and has ridden a bicycle from Land's End to John O'Groats. At the 1989 TOFS Conference in Sheffield, two other adult TOFs spoke; Andrew Wood, the father of three healthy children himself, and David Bland – who as well as playing squash, also rock climbs, hill walks and is a fanatical white water canoeist. Vicki and David n o w r u n t h e i r o w n s u c c e s s f u l businesses. In the TOFS office, letters are regularly received describing the achievements of younger TOFs. Children with TOF and/or OA are harder work in the early years than a

normal healthy child, but after a few years they usually improve to such an extent that for most of the time both the parents and the children forget that anything was ever wrong. The funding for this book has been provided by the family of a TOF child, Benjamin Pouncey – who at the age of 6, having overcome various problems related to the TOF condition, tragically died in a swimming accident. His family set up a memorial fund, to be used by the Tracheo-Oesophageal Fistula Support group (TOFS) for a special project, and it was agreed that the production of a book would be appropriate. This book is therefore dedicated to TOF children and their families, in memory of Benjamin Pouncey, with many thanks to his family and friends.

`Our Son' by the parents of a TOF child Our son was born in '95 Nine weeks early – lucky to be alive Unable to swallow, he was critically ill A major op' followed, we watched over him still Twelve months on, he still c annot eat He's fed by a pump, but we won't admit defeat We long for the day when he'll have his first meal And just can't imagine what rapture we' II feel An anxious year over, he's still tiny in size We care for him hourly, but he is our prize His smile is electric, his heart so strong He's so young and brave – it all seems so wrong M ay ' 96 bri ng hi m only j oy For we love him to bits, he's our special boy

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The TOF Child

Editorial Vicki Martin BSc, BVSc. Although I had seen the occasional TOFS newsletter, the first contact I really had with TOFS was when I was asked to give a talk about my life at their conference. Armed with various slides depicting myself from before my TOF/OA operation through to my recent graduation ceremony, I set out to give a rather light-hearted account of my progress to date. My mother had often spoken of the lack of support she had received in my early years, but it was only through listening to the other speakers and talking to parents attending the conference that I came to appreciate the implications of the problem I was born with and the importance of the TOFS group for families. The presence of a source of advice, support and reassurance does alot to remove much of the sense of frustration, despair and isolation that seems to be all too common. Once the shock of diagnosis has been overcome, many of the difficulties faced by TOF families are of a physical nature e.g. feeding and respiratory problems. This book will help parents by providing factual information about these areas. However, I hope it will also improve the quality of life for TOF families by removing much of the anxiety over the condition – for after all, life is about much more than just survival. Although the early years with a TOF child may bring their challenges, they will also contain plenty of pleasurable moments as in any 'normal' family. If this book manages to facilitate the latter, then my work will have been successful. I have learnt a lot in the course of putting together this book; facts about the

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condition, about other people and about myself. The first was to be expected. The second was a valuable bonus – sharing personal moments with parents and other TOFs, and gaining insight into others' lives and attitudes. The last was in some ways quite unexpected ... as a result of some personal detective work to learn more about the genetic implications of TOF/OA and VACIERL, I requested a referral appointment with the local genetics service. They decided to run a few tests in order to ascertain whether I had any parts of the VACIERL syndrome, and to our surprise we found that I have only one kidney and an extra vertebra and pair of ribs. Neither of these should cause me any future problems whatsoever although it can in certain situations be useful to know that one has only a single kidney and I therefore now wear a "MedicAlert" bracelet. At the time I was born the various tests to check for associated anomalies were not routinely carried out when a diagnosis of TOF/OA is confirmed, as they are now. My thanks and those of the TOFS group go to the medical professionals who have contributed to this book – for without them the publication would not have been possible, especially to Sue Goodley at Nottingham Neonatal Service who helped with the planning of the book as well as giving me the chance to visit her Unit and learn about the initial care of TOF babies. Special thanks must also go to all the TOFS members who shared their thoughts and feelings, and to TOF parent Janita Clamp who read through the many responses and organised them into appropriate sections as well as looking the book over before going to press.


The TOF Child

What is TOF OA? J .A.S . Dickson FRCS, FRCSE, FRCPCH , Consultant Paediatric Surgeon, Sheffield Children's Hospital. The story of oesophageal atresia can reasonably be taken to start with this description from Thomas Gibson's `Anatomy of Humane Bodies Epitomized' published in 1697:About November 1696 1 was sent for to an infant that would not swallow. The child seem' d very desirous of food, and took what was offered it in a spoon with greediness but when it went to swallow it, was like to be choked, and what should have gone down returned by the mouth and nose, and it fell into a struggling convulsive sort of fit but the next day died. The parents being willing to have it opened, 1 took two physicians and a surgeon with me...We blew a pipe down the gullet, but found no passage for the wind into the stomach. Then we made a slit in the stomach, and put a pipe into its upper orifice, and blowing, we found the wind had a vent, but not by the top of the gullet. Then we carefully slit open the back side of the gullet from the stomach upwards, and when we were gone a little above half way towards the pharynx we found it hollow no further we began to slit it open from the pharynx downward, and it was hollow till within an inch of the other slit, and in the imperforate part of was narrower than in the hollowed. This isthmus (as it were) did not seem ever to have been hollow, for in the bottom of the upper, and the top of the lower cavity, there was not the least print of any such thing, but the parts were here as smooth as the bottom of an acorn-cup. Then searching what way the wind had passed when we blew from the stomach upwards, we found an oval hole (half an inch long) on the fore-side of the gullet opening into the aspera arteria* a little above its fast division, just under the lower part of the isthmus above mentioned. * trachea or windpipe

This dramatic account very clearly describes the commonest forms of oesophageal atresia (OA) and tracheooesophageal fistula (TOF).

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The TOF Child

What is Wrong in TOF/OA?

Variations

The normal oesophagus The oesophagus (gullet) is the passage through which food moves on its route from the mouth to the stomach. It starts in the neck, just behind the larynx (Adam's apple), and ends below the diaphragm where it joins the stomach at an acute angle. Oesophageal atresia The word `atresia' is taken from ancient Greek and means 'no passage / no way through.' Thus in oesophageal atresia there is a break in the continuity of the oesophagus. The end nearest the mouth is not attached to the end which enters the stomach, the gap usually occurring high up in the chest. The presence of a blindending pouch in the upper oesophagus means that food is unable to reach the stomach; any s w a l l o w e d mi l k o r s a l i va i n s t e a d returns to the mouth.

In 'An Atlas of Oesophageal Atresia' the German surgeon Dietrich Kluth describes 93 different variants of these abnormalities. Fortunately only five are at all common...

The normal trachea The trachea (windpipe) starts at the larynx (the voice box, seen from the outside as the 'Adam's apple' in the neck) and passes in front of the oesophagus before it enters the upper chest, where it divides into two tubes, the main bronchi, which go to the right and left lungs. Tracheo-oesophageal fistula A fistula, from the Latin meaning 'a pipe,' is an abnormal connection running either between two tubes or between a tube and a surface. In tracheo-oesophageal fistula it runs b e t w e e n t h e t r a c h e a a n d t h e oesophagus. This connection may or may not have a central cavity; if it does, then food within the oesophagus may pass into the trachea (and on to the lungs) or alternatively, air in the trachea may cross into the oesophagus.

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Atresia with lower pouch fistula In 85% of cases the upper oesophagus ends blindly, usually in the upper part of the chest. The lower part starts at the fistula from the trachea, which is located near the point where it divides into the main bronchi. Atresia without fistula In 8% of cases there is no fistula. These babies are often more difficult to treat because the gap between the two ends of the oesophagus is usually very wide and the stomach very small. Fistula without atresia Here the continuity of the oesophagus is not affected, there being no atresia, however there is a fistula joining the oesophagus and the trachea. This variation occurs in about 5% of cases and is often called an 11 fistula,' although the fistula is nearly always oblique and therefore more like an 'N.' Atresia with upper pouch fistula Here the fistula runs from the upper sac of the oesophagus to the trachea. This is one of many variants making up the remaining 2% of cases. Double fistula Occasionally there may be more than one fistula present, with or without an oesophageal atresia. Laryngeal cleft In this very rare condition the noimal separation between the air passage and the oesophagus fails to form, so that food spills over into the windpipe on swallowing.


The TOF Child

Types of Oesophageal Atresia and Tracheo-Oesophageal Fistula

Oesophageal atresia with lower pouch fistula (most common type - 85%)

Oesophageal atresia with upper pouch fistula (less than 2%)

(atresia) Fistul a

-1._ ` No separation of upper airway and oesophagus

Laryngeal cleft

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The TOF Child The Need for Surgery

Diagnosis Pre-

TOF/OA requires surgery for correction of the defect(s); i) the two ends of the oesophagus must be joined together to allow the baby to swallow food such that the nutrients it contains can be digested and absorbed.

natal diagnosis

ii) any connection(s) with the trachea must be closed off to prevent swallowed food / fluids passing from the oesophagus into the lungs – also to stop air passing from the trachea to the oesophagus and then into the stomach. Why Oesophageal Atresia has to be Treated... 1. With an oesophageal atresia, food cannot enter the stomach and therefore the baby has no means of gaining nutrition. 2. Unswallowed fluid may also spill down the trachea into the lungs, causing pneumon a.

Why TracheoOesophageal Fistula has to be Treated... 1. With a tracheooesophageal fistula, air can pass from the trachea to the stomach, causing distension. 2. Food in the oesophagus may also pass into the trachea and lungs, causing pneumonia. Presence of an atresia will affect the relative likelihood of these events.

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Oesophageal atresia may be suspected before birth: i) The inability of the baby to swallow may lead to excess fluid collecting in the womb in 20-25% of babies. This is called hydramnios or polyhydramnios. ii) In those babies with no fistula from the lower oesophagus to the windpipe, the stomach is also very small, there being nothing passing into it to encourage it to stretch and grow. The combination of excess fluid around the baby and a small or undetectable stomach on a prenatal ultrasound scan is therefore strong presumptive evidence that the baby has this rare form of oesophageal atresia with tracheooesophageal fistula. iii) The level of alpha feto protein (used to detect spina bifida and abdominal wall defects) in the mother's blood may also be slightly raised. Under these circumstances, an amniocentesis test may be carried out, to exclude the possibility of a chromosomal (genetic) defect which may be incompatible with the baby's survival; examples of such defects include Trisomy 13, 18 and 21. If these are present the mother should be given the option of an elective termination of the pregnancy. Otherwise, the pregnancy should be allowed to c o n t i n u e t o t e r m a n d t h e b a b y delivered by the normal vaginal route.


The TOF Child

Antenatal Diagnosis of TOF 10A using Ultrasound by Dr Alan Sprigg When babies cannot swallow, excess fluid (known as polyhydramnios) accumulates around them in the womb. This can happen in oesophageal atresia particularly when there is no fistula. A distal fistula allows fluid a route to the stomach by providing a way for it to travel from the trachea to the oesophagus and on to the stomach, bypassing the atresia. Polyhydramnios also happens in severe cases of cleft lip (hare lip) and palate but these conditions can usually be picked up on the scan. Inability to swallow is also seen with brain p r o b l e ms , wh i c h c an n o t b e see n on a scan. The other feature which is seen in scans of oesophageal atresia babies, a very small or absent stomach, has to be noted on several examinations to be significant.

Above: Normal antenatal ultrasound scan, with the baby's head and body, bladder (B) and stomach (S) labelled. The black area immediately above the baby is fluid.

Overall it is not possible to put a mathematical figure on the predictive value of these signs. In the commonest form of oesophageal atresia with tracheooesophageal fistula, even in the presence of polyhydramnios, it is not possible to make an definite antenatal diagnosis by conventional methods.

Right: Two scans of babies who were horn with oesophageal atresia. Both scans show the head to the right as in the normal scan above them. i) Neither scan shows a stomach (compare with the normal scan above). ii) The black area (fluid) around the baby is greater than normal, indicating excess fluid (polyhydramnios).

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Diagnosis after birth

Diagnosis of Oesophageal Atresia

With most TOF/OA babies, there is no warning before birth that anything is wrong. The main characteristic of oesophageal atresia in a newborn baby is the inability to swallow saliva –frothing at the mouth and 'blowing bubbles.' Fluid gaining entry to the trachea and lungs, either through a tracheo-oesophageal fistula or directly from the mouth down the trachea, may cause problems with breathing with the baby making exagerated efforts to breathe and/or showing blueness of the lips and finger tips. Any air passing into the stomach from the trachea via a tracheo-oesophageal fistula (often as a result of the respiratory movements) may also distend the tummy. If an oesophageal atresia is suspected, the passage of a tube down the oesophagus from the mouth will either confirm the diagnosis (if it is held up in the blind-ending sac) or exclude it (if the tube reaches the stomach). It is important that the tube used is a reasonably stiff one; too fine a tube may curl up in the upper pouch and give a misleading result. Radiographs (X-rays) of the chest are essential to see where the tube is being held up and to examine the lungs and heart. Radiographs of the abdomen may also be taken; the presence of air in the stomach means that the lower oesophagus is attached to the trachea (i.e. a tracheooesophageal fistula). Radiographs using barium to further examine the oesophagus are not usually necessary. Once the diagnosis has been confirmed, many surgeons will start the operation by examining the trachea and oesophagus using endoscopy (i.e. bronchoscopy and oesophagoscopy) to identify the length of the upper oesophagus and position of any fistulas.

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In the absence of oesophageal atresia, a tube will pass freely down the oesophagus to the stomach.

If the tube will not pass beyond a small distance from the mouth, an oesophageal atresia is suspected...

...it is very important to use a stiff tube because a flexible one might coil up in an upper pouch, giving misleading results.


The TOF Child Above right: Radiograph taken looking from the side with the baby facing to the left. The jaws and base of the skull is seen at the top, the ribs show as horizontal stripes in the chest, and the vertebrae of the spine appear as blocks to the right. The main features are: i) There is air in the stomach (the dark oval shape near the base of the rib cage). ii) A tube has been inserted through the mouth and down the oesophagus. It has been held up at the site of the atresia. These findings indicate that the baby has an oesophageal atresia and a distal tracheo-oesophageal fistula.

Below left: Radiograph of the chest of a TOF 10A baby, looking from the front. The ribs show as horizontal stripes; the spine and sternum are superimposed in the midline. i) Barium (a so-called 'contrast agent' which appears white on a radiograph) has been given to the baby to swallow; it has collected in the upper pouch of an atresia (a little way down from the top, centrally). ii) The stomach is very distended with air (the large black circle, bottom right).

Above: Radiograph of the chest from the front showing the inadvertant and undesired outcome of a contrast swallow. The contrast agent is in the respiratory tract, highlighting the two main bronchi which branch into smaller airways in the left and right lungs. The contrast agent has also gained access to the stomach however, through a fistula. This is not a standard procedure because contrast agent is not good for the lungs.

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The TOF Child

Conditions Occurring with TOF/OA Other associated congenital abnormalities may also be detected in the abdominal radiograph. The commonest are gut problems either in the rectum (the last part of the large bowel) and/or in the duodenum (the part of the small bowel next to the stomach). These have all been successfully corrected even in very small babies. Rarely all three are seen in the same baby. It is usual to examine the heart and kidneys using ultrasound scanning; defects in these important organs are common because of the associations between TOF/OA and two groups of abnormalities – VATER syndrome (more recently termed VACTERL) and CHARGE. The letters VATER stand for Vertebral (spine), Anal (backpassage), Tracheal, Esophageal (from the American spelling; the UK spelling is `oesophageal'), Renal (related to the kidney) and Radial (the radius bone in the forearm); the extra letters in VACTERL stand for Cardiac (affecting the heart) and Limb (since there are other limb abnormalities other than those involving only the radius). There are separate chapters on VACTERL in this book which give further details. The CHARGE association includes Coloboma (defects affecting the pupil of the eye), Heart disease, Atresia of the choanae (the passages at the back of the nose), Retarded growth, Genital hypoplasia (underdevelopment of the genital organs) and Ear anomalies/ deafness.

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Transfer to Another Hospital The TOF/OA condition requires prompt surgical intervention, so where the diagnosis is suspected before birth it may be decided to arrange delivery in a special maternity unit which is in close contact with a paediatric surgical centre. If this is not possible, it should not cause a problem – for example, many years ago a baby born in Hong Kong was transferred to London for the operation, and arrived in excellent condition. As soon as the diagnosis has been confirmed, transfer to a specialist paediatric surgical unit is arranged, accompanied by suitably qualified staff (this is discussed further in the next chapter). Since the mother is rarely able to accompany the baby, a sample of her blood will be also required for crossmatching before the operation. Parents should be able to see and touch their baby before transfer, and the father allowed to go with the baby in the ambulance. Polaroid photographs are often taken, and copies given to both parents. Arrangements for mother to join her baby depend on her health and how well the receiving hospital is able to look after her; some hospitals are reluctant to look after mothers before the eighth day after delivery.


The TOF Child

On Learning Your Child is a TOF Dr Lorraine Ludman BSc(Hons) PhD, a Chartered Psychologist, is a Research Fellow in the Department of Paediatric Surgery, and Honorary Research Fellow in the Behavioural Sciences Unit at the Institute of Child Health in London. She has worked as a teacher, music therapist, and then as a researcher in psychology at Great Ormond Street Hospital and the Institute of Child Health. Her main research interest has been concerned with infants who require major neonatal surgery for congenital anomalies; she has studied the psychological effects – social, emotional and intellectual, on the developing child, as well as the effects on the family as a whole. Recent work includes a major study of children and adolescents treated for anorectal malformations, and evaluation of therapeutic intervention with children who have anorectal malformations and Hirschsprung' s disease. She also lectures part-time in Developmental Psychology and Health Psychology. The birth of a baby with a congenital abnormality such as tracheoo e s o p ha ge a l f i st ul a / oe s o p h a ge al atresia (TOF/OA), which has not been identified prenatally, and is therefore totally unexpected, comes as a great shock to parents. The first question many parents ask immediately after the birth, is "is the baby all right?" To be told that the eagerly anticipated joyful event – the birth of a perfect baby – has not been fulfilled, is very frightening. Weary and emotionally sensitive after the physical exertion of gi ving bir th, to f ind that the baby needs major surgery, must be like the worst kind of nightmare. This crisis, this 'threat' to their new precious baby, precipitates a range of responses in different individuals – grief, disbelief, dismay, numbness and confusion. At first, many parents find it difficult to understand or take in what was happening. The world probably seems unreal and in a turmoil.

"How can this be happening to me?" "What did I do?" "Who is to blame?" "Will my baby die?" "Will (s)he be 'normal'?" ... are questions voiced or thought about at the time. Despite the turmoil however, decisions have to be made. Because s u r g e r y n e e d s t o b e c a r r i e d o u t urgently and hospitalisation is obligatory, the baby should be transferred to a hospital specialising in neonatal surgery, unless there is a neonatal surgical unit in the hospital where the baby is born. Parents are asked to give consent for the surgery and some may even be asked whether they want to have the baby christened before the transfer.* After the initial shock, the role of each parent generally differs and this carries with it different stresses.

*If specialist treatment is possible in the hospital where the baby is born, parents will not experience all the difficulties mentioned in the next section — however, the infant will undergo surgery soon after birth, and will initially be cared for in an intensive care unit (ICU). Separation of the baby from the parents is therefore inevitable. 15


The TOF Child

Father Depending on her physical state the mother will generally remain in the maternity unit after the baby's transfer. On the other hand the father may well accompany the baby to the specialist hospital. His roles of keeping the mother informed about the baby and giving her reassurance are very important in helping her cope with the mixture of emotions she experiences — loneliness, fear, sadness, anxiety, impatience, self-blame, anger — as well as dispelling any irrational thoughts about the reasons for the baby's defect. On arrival, he may have had the opportunity to meet a member of the surgical team who would have been able to give a general explanation about the baby's abnormality. A more complete assessment of the baby's condition and prognosis has to wait until the results from various investigations become available and the baby has been seen by a senior member of the surgical team. During this initial difficult period the father may have had the added responsibility of looking after the couple's other children, or making arrangements for them to be looked after, as well as taking care of household and family matters. It is quite common for the effects of the emotional upheaval and the daily stresses and strains that fall upon fathers to be underestimated and overlooked. Moreover, the father may feel it is unmanly to express his feelings and anxieties and may be reluctant to discuss them either with his partner or with family and friends.

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Mother After the baby's transfer many mothers have to come to terms with being without their baby. They may b e come ver y a nxi ous an d har bou r secret fears — will they be able to love their baby despite his or her faults? Will they fail to 'bond' because they have been separated from their baby? They should be reassured however. Contrary to a widespread emphasis in the popular press and academic literature in the 70's and 80's on the importance of bonding and attachment — an 'instantaneous gluing' which was thought to happen in the first few minutes after the birth — there is no strong supporting evidence that this o c c u r s , o r i n d e e d i s a u s u a l o r necessary response for the development of a loving mother-child relationship. We know that the development of the bond with the baby usually occurs over the course of time, and that this may va r y f r o m a f e w h o u r s o r da ys t o several months. Moreover, separation by itself is not likely to be the cause of disturbed motherchild relationships.


The TOF Child

Coming to Terms with Reality After the initial shock, and once the mother is over the delivery and is able to visit the baby, life does not necessarily return to normal. Having to leave the maternity unit and return home without her baby is a situation which can be very traumatic for the m o t h e r . H e r c i r c u m s t a n c e s a r e contrary to the expectations of her friends and family and she may find she is involved in trying to explain a situation which she herself does not fully understand. This can become tiresome and distressful. Relationships with those close to her, as well as with acquaintances, can become strained. Few can understand the situation the parents are facing or the emotions the parents are experiencing. Moreover, each parent will react differently to the `crisis.' However close their relationship was before the birth, they may find it difficult to understand their partner's behaviour and reactions. Being able to talk to each other about t h e i r f e ar s a n d a n xi et i e s i s o f t en helpful in overcoming feelings of isolation and stress. This can however take time, and may require a lot of patience and mutual understanding.

Post-Operative Period Many mothers have told me that during the early weeks after the initial surgery, they functioned as if in a dream. Their waking hours were often spent in the intensive care unit or wherever the baby was being cared for, and the world around them appeared unreal. In addition, hospitals and the hi-tech environment of the intensive care unit were very frightening for some parents. Getting to sleep was often very difficult, and in general they appeared to be existing in a haze of

constant anxiety. Some mothers told me that they felt guilty. They found it very boring and depressing having to spend most of the early days and weeks after the initial operation(s) sitting by their baby's cot. In spite of this, they felt that they had to do what was expected of them and were afraid to discuss these feelings, in case they might be considered potentially poor and neglectful mothers. On the other hand, some mothers became distressed if they were not able to be with their baby all the time, and were not given enough opportunity to participate in their care. A number of mothers told me that sleeping at home in their own beds helped them to feel more secure and more in touch with reality. If they lived far from the hospital, and had chosen t o s t a y i n o r n e a r t h e s p e c i a l i s t hospital, the ability to return home at least once or twice a week was benefi cial. Conver sel y t her e were those who would not be apart from the baby; if the baby was in hospital for several weeks or even months, this can put quite a strain on the parental relationship. The father might begin to feel left out and feel that his needs (both emotional and physical) were being ignored. Nevertheless, it is wise to remember that this particular problem (a mother's preoccupation with her baby) is not unique to parents with a sick baby, since similar problems occur after the birth of a healthy child. Many fathers will return to work shortly after the birth. For some, this will help as they will be distracted from dwelling on the baby's problems. For others, it will just be a further burden which adds to an already stressful situation. Those who live some distance from the hospital may

17


The TOF Child find it impossible to visit the baby except at weekends. Consequently the father may begin to feel somewhat remote from his partner's problems and anxieties; this can increase feelings of guilt and be an additional cause of strain between the parents. In conclusion, coping with a crisis of this nature and magnitude will affect individuals in different ways. Responses will be determined by a multitude of factors such as whether they are the mother or the father, their age, family and social circumstances, the way they react to stress, and the severity of the baby's condition. During the early traumatic stages, it may help to know that, given time, the nightmare fades. As the baby recovers from the operation(s) and is able to go home to be cared for by his or her parents, a relatively `normal' pattern of family life will, in the majority of cases, slowly become established. Although there may be many crises still to come, many of the parents interviewed told me that by the time the baby was 10-12 months old they could start to compare their baby with others who had not had such a dramatic introduction to life, and begin to feel less afraid for their infant's future development.

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Prenatal Diagnosis In the last few years, it has become possible to identify a TOF baby before birth at the time of ultrasound scanning. Consequently, a small number of parents may have prior warning that all is not well with their baby and that he or she may have OA. Mothers who develop polyhydramnios in later pregnancy may also be aware of an impending abnormal infant. How do parents cope with this? To some extent, this may depend on whether the TOF appears to be an isolated problem, or part of a constellation of anomalies. With support and advice from very well informed professionals, and possibly families who have been in similar situations, these parents will have time to come to terms with the idea that the baby will not be 'perfect.' This period may also provide time to understand and acknowledge some of the difficulties that lie ahead. Since a high proportion of TOF babies have a very good chance of surviving the birth and subsequent corrective surgery, parents may be less anxious, and await the birth relatively calmly. The manner in which individuals cope with stress of this nature

will however also depend on their personality, social situation and the quality of support from those close to them. Prenatal diagnosis may also mean that the birth may be prearranged to take place at a specialist centre, overcoming any need for transfer of the baby after birth.


The TOF Child Quotations from TOFs, their families and friends and medical professionals "The most vivid memory of those blurred early days was when Jacob w a s wheeled to theatre in his incubator – so fragile and vulnerable, and us as parents holding each other and sobbing feeling helpless and useless. I cry even now to think of it." "To see doctors and nurses in the b ack gro un d run ni n g ar ou nd yo ur small child I think really brought home what was really happening to us and to Jamie as up until then I think we were wandering about in a daze for the first 4 – 5 weeks, as we were travelling around 60 miles per day to the hospital. I think all the running around you have to do doesn't give you any time to think about what is happening." "I have never really felt 'why me?' I believe about 1 baby in 3,000 has problems like Colin' s, but statistics are meaningless when your baby is the one. I did feel angry when I had to go into the day room where smoking was allowed, to find a phone. I had been so careful when I was pregnant – yet my baby had problems, and here were these women puffing away, whose babies were healthy. It didn't seem fair. It also annoys me, even now, if parents say they were disappointed to have a girl instead of a boy, or vice versa. I say, as politely as possible, that they should be glad to have a healthy baby." "Nicholas' older brother has a special relationship with his granny who he had to live with for some time. He also feels left out at home because of the attention given to his brother."

"One of the nurses said to me "Don't worry – we get pl ent y of TOFs i n here." Strange as it seems now, at that time I had never heard the term TOF,' I had just been told there was a pouch in Colin' s oesophagus. So I thought she had said 'We get plenty of tofs in here' , meaning that they treated a lot of rich kids and were therefore well-trained in giving of their best. I thought this was a very odd thing to say, and replied Tut he's just an ordinary little b o y ' w h i c h s h e m u s t h a v e f o u n d strange. It wasn't until several days later that I realised what she had said." "The initial elation of an apparently normal home delivery changed by the next day to shock, anxiety and sorrow as severe problems presented and prognosis for Nicholas was poor. The major surgery over many weeks seemed incredible for such a tiny baby. The courage and support that Mary and Paul gave to each other and the continued care of Timothy (aged 3) was a real example to all the family as they waited to discover what the future held." "I was frightened to begin with as the child was so ill after birth. As I found out more about the condition I became less worried, but those first few months were terrifying." "My first reaction was horror and panic. When Nicholas' physical internal problems were explained to me I did not expect him to survive. I was amazed at what was surgically done for his repair and that it was successful." "The quote I remember from hospital during his initial stay was "two steps forward and one step back," at times it seemed the numbers were reversed."

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"Of course we were all devastated when Thomas was born to find that he had lots of problems. The ch ildren were very upset, particularly Ben (8). He suffered nightmares for about four months after Thomas was born. Sonja (6) was much more resilient. It took Ben a week or two before he would hold Tom, but Sonja was there just as soon as possible — in hospital, holding Tom with all his tubes. Ben and Tom are very close now. Ben is away at Cardiff University and Tom misses him dreadfully." "We were very worried and afraid for Jordan's life. We were horrified by him not having a complete gullet and wanted to know why our grandson? Why our daughter? How?"

Above: Tom Dobbs awaiting surgery.


The TOF Child

The Management of Professor L Spitz PhD FRCS, Nuffield Professor of Paediatric Surgery, Institute of Child Health (University College London) and Great Ormond Street Hospital for Sick Children, London. Specialist Information: Transfer of the TOFIOA baby Following delivery of the baby it is essential that the airway is kept clear of secretions at all times and that aspiration pneumonia is prevented. This is best achieved by passing a large calibre naso-oesophageal tube of the Replogle type to which continuous suction is applied. If this type of catheter is not available then a similar sized nasogastric tube should be passed into the upper pouch and it should he intermittently aspirated using a hand syringe. The baby's mouth should also be kept clear of secretions by intermittent aspiration of the oral cavity. The best position for the infant during transfer is supine or on the right side. The baby must he kept warm at all times and transferred in a portable incubator, accompanied by a qualified nurse experienced in caring for small, at risk infants. Vitamin K by injection may be given at the referral hospital but if administered this fact must be recorded in the transfer notes and referral letter Fluid administration in the, first few hours of life is not a requirement. If the baby is premature and/or has breathing difficulties, such as respiratory distress syndrome, mechanical ventilation may be required during transfer. This must he carried out with extreme care as it may result in distension of the stomach which will further impair respiratory problems. It is clearly in the best interest of the baby that transfer should take place to a large neonatal surgical unit which is staffed by experienced paediatric surgeons and by fully trained paediatric intensive care nurses. Units which regularly treat infants

TOF/OA TOF/OA

Pre-Operative Management Emergency surgery for the infant with oesophageal atresia is no longer necessary. Indeed, a period of preoperative stabilisation before submitting the baby to a major operation will facilitate the postoperative recovery. The infant 's temperature should be kept within the normal range and any fluid or electrolyte imbalance should be corrected before surgery. The operation can safely be postponed until the daylight hours rather than having to be carried out in the early hours of the morning when the surgeon may not be at his or her best, when theatre staffing is at emergency levels and when the ward may have a reduced complement of experienced nurses. However, the lack of availability of a regular operating theatre may dictate that the surgery has to be undertaken at night rather than waiting until the following evening. The only clear exception to this is the infant who has respiratory difficulties. It is particularly the premature infant with respiratory distress syndrome who is at risk. The baby may require mechanical ventilation because of the "stiff lungs" and in the presence of a distal tracheooesophageal fistula, the high pressures necessary to-keep lungs inflated may cause a significant proportion of the inspired gases to escape down the fistula into the stomach. The stomach becomes increasingly distended, worsening the respiratory status, and

f

21


The TOF Child the stomach itself may eventually rupture resulting in a catastrophic situation which is immediately lifethreatening. We have found that the best method of managing this sequence of events is to undertake an emergency operation to ligate the fistula. If the infant's respiratory status dramatically improves, the surgeon may be happy to continue with the p r o c e d u r e , b u t m o r e u s u a l l y t h e infant's condition is so unstable that the operation is best left at this stage and the repair postponed for a few days when the problem with the lungs should have resolved. Another circumstance which may delay surgery or dictate a different approach is where the infant has a major congenital heart abnormality. Detailed investigations of the precise anatomy of the heart will be required (using echocardiography) and medical treatment for cardiac failure instituted. In rare circumstances, where medical treatment is unsuccessful, cardiac surgery may be advisable before correction of the oesophageal atresia in order to give the baby its best chances of survival.

Surgery: Primary Anastomosis

Passage of gases into stomach during mechanical respiration in a TOF baby with stiff lungs due to respiratory distress syndrome

Upper pouch (atresia)

Gases from mechanical respiration pass into stomach

Fistula

Enlarged stomach Risk of stomach rupture

Alleviation of pressure in the stomach by ligation of the fistula

Upper pouch (atresia) Fistula 'tied off'

Gase s confined to airways

The aim of surgery for oesophageal atresia is to divide the tracheooesophageal fistula and to perform a `primary anastomosis,' joining the two ends of t he oesophagus dur ing the initial operation. Occasionally, primary anastomosis will not be possible at the first operation because the gap between the t wo ends of the oesophagus is too wide. Surgery in these cases is described later in this chapter, under long-gap oesophageal atresia.'

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Preparation Following pre-operative assessment and stabilisation, the infant is prepared for the operative correction of the oesophageal atresia. Consent for the procedure is obtained and the risks of the operation and the chances of the baby's survival are carefully explained to the parents.


The TOF Child We have recently proposed a new classification scheme whereby if the infant's birthweight is greater than 1500 grams and there is no evidence of a major congenital heart anomaly, the chances of survival are close to 100%. If the baby weighs less than 1500 g r a m s a t b i r t h o r h a s a m a j o r congenital heart anomaly, the chances of survival are reduced to 60%. If the baby's weight is below 1500 grams and there is a major heart anomaly, the chances of survival are much further reduced to around 20%. The baby is then taken to the operating theatre where anaesthesia is induced by an experienced paediatric anaesthetist. The procedure is carried out under general anaesthesia with continuous and close monitoring of all vital functions (i.e. heart rate, electrocardiogram, blood oxygen saturation level, body temperature).

Incision The usual incision is along a curved line on the right side of the chest close to the back to just under the tip of the shoulder blade. It should not extend close to the nipple.

Positioning and Incision Site for Surgery

Line of incision

Arm placed alongside head

Surgery for TOF/OA Division of tracheooesophageal fistula

Oesophagus Trachea

Closure of tracheal end of fistula: tube in upper pouch

Sutures being placed for the anastomosis between the upper pouch and distal (lower) oesophagus Continuity restored: transanastomotic tube inserted

Diaphragm Shoulder blade

Procedure The chest is entered between the fourth and fifth ribs and without opening the membrane which encloses the lung, the area of the oesophageal atresia is approached. The distal tracheooesophageal fistula is

Transanastomotic tube Stomach

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The TOF Child carefully identified and traced towards its entry into the trachea, where it is divided and the defect in the trachea closed with fine interrupted sutures. The proximal (upper) oesophagus is now identified with the help of the anaesthetist applying pressure on the tube in the upper oesophageal pouch. The upper pouch is mobilised sufficiently to allow anastomosis (joining) to take place with the least possible tension. If there is significant tension at the anastomosis, the infant may be electively paralysed and mechanically ventilated (i.e. put on a life-support machine') to reduce the likelihood of a leak in the anastomosis. The anastomosis is performed using a single layer of full thickness fine interrupted sutures. The anastomosis should be watertight.

Transanastomotic tube Most surgeons will pass a transanastomotic tube (trans = across; anastomosis = join) from the nose through the oesophagus into the stomach to remove any pressure in the stomach during the first few days after the operation, and to allow feeding to o c c u r o n t h e t h i r d o r f o u r t h postoperative day.

Chest drain Some surgeons leave a chest drain in position for up to one week, others have omitted this as it does not appear to be of any great value in detecting an anastomotic leak (a defect in the join that allows oesophageal contents to leak into the surrounding tissues).

Closure The wound is closed in layers, with a subcuticular suture to the skin (a stitch that is buried in the skin, underneath the surface). This type of stitch does not require removal.

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Long-gap Oesophageal Atresia Sometimes the gap between the two ends of the oesophagus is too large to bridge immediately (long-gap oesophageal at r esia') and i n t hese cases a gastrostomy is performed. A gastrostomy is a tube placed through the wall of the abdomen (on the left side) and passes into the stomach to allow the baby to be fed. After dividing the TOF, the distal ( l ow er ) oes op ha gus i s cl o sed of f , unless it is already blind, and a tube left in the upper pouch which is either left attached to a suction machine or manually aspirated at regular intervals, to prevent overflow of saliva into the lungs. This latter tube needs careful and meticulous nursing care. The gap between the upper and l o w e r e n d s o f t h e o e s o p h a g u s i s assessed radiologically at around six weeks to ascertain whether a delayed primary anastomosis is feasible. A d el a yed p r i mar y an as t o mo si s i s a direct joining of the two ends of the oesophagus which has been delayed to allow the oesophagus a little further time in which to lengthen.


The TOF Child Gastrostomy Tube

Upper pouch (atresia) with tube inserted

Gastrostomy tube

Front surface of the stomach is sutured to the abdominal wall

A further radiological study may be carried out at 12 weeks but delaying further than this is unproductive and the child should be submitted to an oesophageal substitution procedure rather than persisting with the intention of perfot ining a delayed primary anastomosis after 12 weeks (see next chapter). If the gap between the ends of the oesophagus is excessive (for example, greater than the length of six vertebrae when viewed on a radiograph), the surgeon may decide not to wait the six to twelve weeks before reassessment as described above, and will instead proceed immediately to the procedures related to oesophageal substitution.

Stomach

Above: Detail of gastrostomy tube showing how the tube is attached

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The TOF Child

Oesophageal Substitution Procedures Professor L Spitz PhD FRCS, Nuffield Professor of Paediatric Surgery, Institute of Child Health (University of London) and Great Ormond Street Hospital for Sick Children, London. There are occasions when a primary anastomosis (join) of the oesophagus, either immediate or delayed, may be considered an impossibility due to the gap being too wide. Under these circumstances the baby is encouraged to thrive until old enough (around 6-9 months) to undergo an oesophageal substitution procedure. This is an operation in which an alternative part of the gastro-intestinal tract is utilised to bridge the gap between the two ends of the oesophagus, so substituting for the normal oesophagus as a 'pipe' between mouth and stomach. During the months before this can be carried out, the baby needs to gain the experience of food in his/her mouth, to learn to chew and swallow, and to associate that with the feeling of satisfaction at having been fed. Learning these actions and associations later on, after replacement sur ger y, is ver y much harder to achieve. It is therefore necessary to bring the end of the upper oesophagus out in the neck as a cervical oesophagostomy and to give the infant a feeding gastrostomy. If there is an associated tracheo-oesophageal fistula, it will require division and closure. Feeding is then provided by the gastrostomy and the baby is encouraged to suck and take small amounts of feed by mouth (`sham feeding,' described in the next chapter) even though this will not provide any 26

Cervical Oesophagostomy and Gastrostomy

Sham feed

Cervical oesophagostomy

Gastrostomy tube

nourishment and will soil the neck at the site of the oesophagostomy. Failure of the baby to sham feed can cause major swallowing difficulties to occur when the oesophageal substitution is eventually carried out. Surgery for additional abnormalities may be necessary around the time of the birth. These may be correction of a duodenal atresia or an anorectal malformation where either a colostomy is performed or a perineal operation is carried out to create an anal orifice.


The TOF Child There are various options for oesophageal substitution surgery: i) colonic interposition ii) gastric tube oesophagoplasty iii) gastric transposition iv) jejunal interposition v) g a s t r i c e l o n g a t i o n The first three are the most widely used. Jejunal interposition is rarely used because of the precarious blood supply to the interposed jejunal segment. Gastric elongation by dividing the lesser curvature of the stomach has been proposed by Scharli but is not widely employed.

Colon Interposition A section of colon is taken from its normal location in the gut, and transposed with its blood supply intact into the chest, where it is joined to the oesophagus above and the stomach below. The Gastro-Intestir Tract with Long-Gap OA

This is still the most widely used procedure.

Advantages i) The length of the graft required is not a problem, unlike other options. ii) Provides a tube of a good diameter.

Disadvantages i) Blood supply to the transplanted section of colon is precarious. ii) Poor peristalsis (the muscular action which transports food down the tube). iii) High incidence of leakage (30% of patients). iv) Stricture (narrowing) can occur (20% of patients). (v) Redundancy (lack of any muscular activity) can develop long term. Colon Interposition Colon

A section of colon is removed from its usual situation, together with its blood supply...

Blood supply

Upper end of oesophagus Oesophagus (gullet)

Sutures

Transposed colon

Sutures Diaphragm Lower end of oesophagus

Duodenum

Blood supply to colon

Jejunum and ileum Colon Caecum (appendix)

•

Rectum Anus

Stomach iv

It is then placed in position, between the two ends of the oesophagus, and sutured in place.

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The TOF Child

Gastric Tube Oesophagoplasty A longitudinal segment is taken from the stomach, which is then swung up into the chest and joined to the oesophagus.

Advantages i) Size of the graft is appropriate. ii) Good blood supply.

Disadvantages i) Very long suture-line. ii) High incidence of leakage (70% of patients). iii) High stricture rate (50% of patients). iv) Reflux commonly occurs.

Gastric Transposition The whole stomach is freed, mobilised and transposed into the chest. The upper end of the oesophagus is then anastomosed to the top of the stomach in the neck. This is a relatively new technique and its long-term effectiveness is therefore not yet proven.

Advantages i) Excellent blood supply. ii) Incidence of leakage and strictures both reduced to 6% of patients. iii) Relatively simple procedure.

Disadvantages i) Poor gastric emptying. ii) Bulk of the stomach is in the chest; the sheer volume of this affects breathing so that respiratory capacity is reduced. iii) Reflux can be a problem. iv) 'Dumping' occurs when food enters the intestine quite quickly. It causes sweating, dizziness and diarrhoea. It usually lasts only a few months and then disappears.

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The TOF Child

Post-Operative Care

Outlook

Oesophageal substitution, irrespective of the technique used is a major operation requiring technical skill, expert anaesthesia and high level intensive care post-operatively. Elective paralysis and mechanical ventilation (using drugs to inhibit breathing so that respiration has to be taken over by machines) for a few days post-operatively are generally recommended; this puts less stress on the suture lines and therefore gives them a better chance to heal. The commencement of oral feeding may be a difficult and prolonged process, particularly if sham feeding was neglected. For colon interposition and gastric tube oesophagoplasty patients, gastrostomy feeds can be used as a 'backup' until oral feeding is established. For gastric transposition patients, the stomach itself has been moved, so a gastrostomy tube is not an option. A jejunostomy tube (a tube into the jejunum, which is a part of the gut a little way below the stomach) is therefore usually inserted for feeding purposes.

Unfortunately, long-term complications must be anticipated in all replacement procedures. For colonic interposition, strictures at the anastomotic site early on, and redundancy and food stasis in the long term are well known problems. The gastric tube oesophagoplasty is prone to stricture formation and reflux of acid content into the upper oesophagus. This may produce inflammatory changes (Barrett's oesophagitis). The gastric transposition may be complicated by delayed gastric emptying and dumping in the short term, and iron deficiency in the long term (probably caused by food moving swiftly through the part of the gut where iron absorption occurs, so that there is little opportunity for uptake). Should the chosen technique for oesophageal replacement fail or be unsuitable, one of the alternative methods should be implemented. Reduced respiratory function is a feature of all oesophageal replacements but generally the children cope extremely well. Left: After a primary anastomosis failed, Emma Louise Dodd was given a cervical oesophagostomy and gastrostomy, and was sham fed for many months before a colon interposition was performed. She has also undergone surgery for scoliosis. She is shown here aged 19 years, a University student who lives a full and happy life. Swallowing is not entirely trouble-free, however she has been discharged from all the hospitals she was attending.

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The TOF Child

The Post-Operative Care of Children with TOF/OA RG Buick, Consultant Paediatric Surgeon and AD Lander, Senior Lecturer in Paediatric Surgery, Birmingham Children's Hospital NHS Trust. Most babies with OA and TOF will be operated on within the first few days of life. Before the operation, some parents have the opportunity to see the ward where the baby will be recovering and to meet the key medical staff who will be involved. In this case, much of what will happen after the operation can be explained in advance. However, it is not uncommon for a baby to be transferred to a more specialised hospital, many miles from the one in which they were born and where the diagnosis came as a surprise shortly after birth. In many hospitals the condition may be seen only once a year — or less — making it difficult for the staff there to answer all the parents' questions. Parents are often unable to accompany their baby to the new hospital; the mother will still be recovering from the delivery or perhaps from a caesarean section, and the father may wish to stay with the mother or be required to look after other children in the family. So for many parents, the first time they see their baby after his/her transfer is after a major operation; by this stage, the baby will be on a specialised ward which is staffed and equipped to look after little babies. Seeing their baby in this high-tech environment can be stressful for parents. The ward may be called the Intensive Care Unit (ICU), Intensive Therapy Unit (ITU), Special Care

Baby Unit (SCBU) or it may just have its own name, unrelated to its function. These wards look after those babies who are very ill or who have had major operations. Many of the babies are quite small and may have been born prematurely. Initially, the ward can be intimidating and frightening with all the equipment, noises and flashing lights, and may seem unfriendly because of the busy staff, lack of space and the limits on the number of visitors. However, parents will soon find that they are welcome. There will be one specific nurse looking after the baby during any one shift; he or she will be able to explain much about the baby's care and progress, and can also introduce parents to the routine of the ward.

Monitoring Immediately after the operation, the baby needs close monitoring and frequent care. Usually a baby is nursed on a special high table, with sides and an overhead heater. Most babies have their heart rate, temperature and blood oxygen saturation level monitored. There may be a heart tracing known as an ECG (electrocardiogram) which is displayed on a screen; this is obtained through thin wires attached to the arms and chest. The blood oxygen saturation level is monitored from a small device applied to a hand or foot and connected by a narrow cable to a

30


The TOF Child

The Post-Operative Care of Children with TOF/OA RG Buick, Consultant Paediatric Surgeon and AD Lander, Senior Lecturer in Paediatric Surgery, Birmingham Children's Hospital NHS Trust. Most babies with OA and TOF will be operated on within the first few days of life. Before the operation, some parents have the opportunity to see the ward where the baby will be recovering and to meet the key medical staff who will be involved. In this case, much of what will happen after the operation can be explained in advance. However, it is not uncommon for a baby to be transferred to a more specialised hospital, many miles from the one in which they were born and where the diagnosis came as a surprise shortly after birth. In many hospitals the condition may be seen only once a year – or less – making it difficult for the staff there to answer all the parents' questions. Parents are often unable to accompany their baby to the new hospital; the mother will still be recovering from the delivery or

Baby Unit (SCBU) or it may just have its own name, unrelated to its function. These wards look after those babies w h o a r e ve r y i l l o r w h o h a v e h a d major operations. Many of the babies are quite small and may have been born prematurely. Initially, the ward can be intimidating and frightening with all the equipment, noises and flashing lights, and may seem unfriendly because of the busy staff, lack of space and the limits on the number of visitors. However, parents will soon find that they are welcome. T h e r e w i l l b e o n e s p e c i f i c n u r s e looking after the baby during any one shift; he or she will be able to explain much about the baby's care and p r o g r e s s , a n d c a n a l s o i n t r o d u c e parents to the routine of the ward.

perhaps from a caesarean section, and the father may wish to stay with the mother or be required to look after other children in the family. So for many parents, the first time they see their baby after his/her transfer is after a major operation; by this stage, the baby will be on a specialised ward which is staffed and equipped to look after little babies. Seeing their baby in this high-tech environment can be stressful for parents.

Immediately after the operation, the baby needs close monitoring and frequent care. Usually a baby is nursed on a special high table, with sides and an overhead heater. Most babies have their heart rate, temperature and blood oxygen saturation level monitored. There may be a heart tracing known as an ECG (electrocardiogram) which is displayed on a screen; this is obtained through thin wires attached to the arms and chest. The blood oxygen saturation level is monitored from a small device applied to a hand or foot and connected by a narrow cable to a

The ward may be called the Intensive Care Unit (ICU), Intensive Therapy Unit (ITU), Special Care

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Monitoring


The TOF Child nearby unit with a screen which also displays the baby's pulse rate. There may be a thin wire attached to a foot to monitor body temperature.

Above: Oxygen saturation and heart rate monitors.

To keep the upper oesophagus clear of saliva immediately after the operation, suction may occasionally be applied at a measured distance from the mouth, so that the inserted tube does not damage the sutures at the join or anastomosis.

Infusions or Drips There may be a number of infusions of fluid running through tubing to one or more special needles (cannulas) in the baby's veins. The cannulas are usually sited on the backs of hands or feet, in the arms or on the scalp and are secured with tape. Once inserted they are not painful. When fluid is running through the cannula into the vein it is Below: Jacob Mayo, aged 3 days. This picture shows the monitoring leads and infusion lines which are commonly used post-operatively. 1. Heart rate and breathing monitors are seen on the chest. 2. The oxygen saturation measuring probe is on the baby's right hand. 3. Intravenous infusions are attached to the right arm. 4. There is a chest drain tube leading away to the bottom right. 5. The baby is attached to a respirator. 6. There is a trans-anastomotic tube.

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The TOF Child commonly known as a 'drip' – a term more applicable to the equivalent system in adult patients, where fluids are usually delivered from bottles or 'packs' of fluid which drips down into tubes. In infants, fluid administration is now nearly always controlled by more accurate electronic pumps; there may be a collection of these small pumps attached to a stand near the cot. The infusions may carry drugs (e.g. antibiotics and drugs for pain-relief) as well as the essential fluid and salts (electrolytes) that the baby's body requires.

Ventilators Some babies may need help with their breathing; this is supplied by a ventilator. Certain babies may have been on a ventilator before the operation – even before transfer – perhaps because of immature lungs or aspiration pneumonia (inflammation in the lungs which results from milk spilling into the lungs; this may have happened during the first attempts to feed, from which the oesophageal atresia was suspected). In the latter situation, it is likely that the baby will still need to be ventilated for a period after surgery, however many babies who were not ventilated before the operation will be ventilated afterwards for anything from a few hours to a few days. Some surgeons choose to paralyse (i.e. give a drug which prevents movement and breathing effort) and ventilate babies for a period of five days if there is tension on the repaired oesophagus. The ventilator is usually contained in a unit on a stand near the cot. It is connected to an oxygen supply on the wall and by two plastic tubes to the baby.

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The attachment to the baby is usual l y t he t hi ng t hat f i r st st ri kes parents when they see their baby after an operation, because the two tubes and their attachments lie over the baby's face and forehead. One of these tubes carries oxygen to the baby; the other takes away the gas which is breathed out. A smaller tube joins these two larger tubes to the baby's airway (trachea) though the nose or mouth. The ventilator makes a cyclical blowing noise as it pushes oxygen-enriched air into the lungs; the tubes to the baby often move in time with this as the pressures within them change. If the baby has been paralysed this action may repeat between 20 and 60 times a m i n u t e , b u t l a t e r o n a s t h e b a b y "wakes up" and makes some efforts at breathing, the ventilator may help with breaths as little as 5 times a minute. Ventilated babies usually have an "arterial line" which allows the oxygen Below;

Eleanor Butcher, 3 days with parents looking on. This photograph shows the two tubes leading from the baby's mouth to the ventilator.


The TOF Child and carbon dioxide levels in arterial blood to be measured from samples taken every few hours. Blood samples taken in this way are painless and the results allow the ventilator to be adjusted appropriately. The arterial line consists of a cannula which has been inserted into an artery and is attached to an infusion pump. Sometimes a blood pressure monitor is also attached to the line and the results displayed on a screen.

Chest Drains The most common operation to repair a TOF/OA involves dividing the t r a c h e o o e s o p h a g e a l f i s t u l a a n d joining the upper and lower parts of the oesophagus. This procedure is performed through the chest (as described in a previous chapter). Some surgeons leave a plastic tube in the chest to drain any fluid or air that might otherwise build up in the space between the lung and the chest wall. If the join in the oesophagus leaks, this fluid will also come out through the drain. The drain is attached to a fat rubber tube that is secured to the cot/incubator and passes to a large bottle on the floor. The bottle contains water, and the end of the tube lies below the level of this water, providing an underwater seal to the tube that allows fluid and air to escape from the chest, but not get back in. The drain may be left in for just a few days or until a contrast radiograph (which uses a special dye in the oesophagus) has shown that there is no leakage from the join, usually at about 7 days after the operation. When the time comes, the tube is easily removed. The hole closes and heals well without further intervention and leaves only a small scar.

The Chest Wound The incision in the chest is usually on the baby's right side, below the shoulder blade, and will have been closed with dissolvable sutures that lie under the skin. The wound may or may not have a dressing; at first the wound may be a little red but this settles down.

Feeding The oesophagus will have been repaired around a plastic tube 3 or 4 mm in diameter. This tube allows milk to be given, either continuously or as small, frequent meals. The tube passes from the nose to the stomach and is c a l l e d a n a s o ga s t r i c o r N G t u b e ; because the tube passes through the place where the oesophagus has been joined, or anastomosed, staff may also call it a transanastomotic tube. Occasionally surgeons may remove this tube immediately after the operation. Feeding is started anywhere between 2 and 7 days after the operation. Some surgeons allow this to be by mouth from the very beginning – others wait until full feeds have been achieved via the nasogastric tube. If a chest drain has been inserted, feeding by mouth may be delayed for 7 days until the join in the oesophagus has been shown to be water tight on a contrast radiograph. Small or sick babies and those with other problems or abnormalities may not be able to feed so promptly or by mouth. Sometimes the surgeon will have placed a gastrostomy tube during the operation, which passes into the stomach through the front of the abdomen and can be used for feeding. Intravenous feeding, known as parenteral nutrition – or sometimes total parenteral nutrition (TPN) – is also used. This may be given through a short

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The TOF Child cannula in a limb or through a long, very fine feeding line which has been threaded up a vein in the head, neck, groin or in a limb into a big vein near the heart. The advantage of these "long lines" into bigger veins is that more concentrated nutrition can be given than is possible through normal "drip" lines.

recovery are made at the referring hospital near the parents' home. When discharged from hospital, arrangements are made for the surgeons to see the baby in the outpatient department to review progress.

Other Problems Leaks

Below: Kris Spender, 19 weeks, being fed. The tubes under the nose give the baby supplemental oxygen.

A major leak of fluid from the join in the oesophagus is now an unusual event thanks to modern surgical advances, but remains a serious one. If a leakage occurs, it usually does so in the first three or four days after the operation. A leak may be suspected because the baby becomes ill with a fever, and may require more oxygen or ventilation. A chest radiograph may show some air or fluid around the lung. If there is any doubt over the diagnosis a small amount of liquid w h i c h s h o w s u p c l e a r l y o n a radiograph can be put into the oesophagus and radiographs taken to see whether this appears to leak around the anastomosis area at all. A small leak may be treated with antibiotics and stopping the feeds by mouth or tube. A larger leak, which the surgeons believe will not heal up on its own, will require surgery.

Tracheomalacia

Recovery As progress is made the baby will require fewer tubes and less frequent monitoring. He or she may move to a more normal-looking cot, and may even move to a less intensive ward while feeding is established. Most babies are ready to go home after 7-10 days of satisfactory feeding on the breast or by bottle, but others may take longer because of prematurity, other anomalies or feeding / breathing problems. Every baby is different. Sometimes the last few days of

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When a baby is born with tracheooesophageal fistula, the trachea is frequently not as ridged as normal. Normally the trachea is held open by stiff rings of cartilage which stop it collapsing when the baby breathes; these rings play the same role as the stiff rings that keep the hose pipe open on vacuum cleaners. Any area lacking these supporting rings is likely to be a bit floppy and may narrow during breathing. This narrowing may be a minor problem that the baby grows out


The TOF Child of – however, sometimes when the baby is distressed and crying the floppy trachea narrows so much that the baby cannot breathe and goes blue. If these episodes are considered severe and/or often enough to be dangerous, later surgery is often recommended (see chapter on respiratory problems).

Jaundice The baby may become yellow, or jaundiced, during the first week of life. This is not uncommon in otherwise noinial newborn babies. This yellow colour should gradually fade and disappear without treatment; if the jaundice increases to a certain level, a special ultraviolet light will be shone on the baby to reduce the jaundice.

Gastro-oesophageal reflux This is another common problem seen with oesophageal atresia. In this condition, milk returns back up the o e s o p h a g u s a f t e r i t h a s b e e n swallowed. Sometimes stomach acid is also forced up the oesophagus, where it can cause inflammation and scarring (see chapter on Strictures and Reflux).

Quotations from TOF s, their families and friends and medical professionals "One of the amazing things was seeing how quickly tiny babies can recover and heal after the kind of major surgery which would render adults incapacitated for months. During Hannah's first week, there were times when my feeling of separation from Hannah was overwhelming, particularly at night when I could hear other babies crying and I was alone in my room. I often found I naturally awoke when Hannah was due her "all care" every 3 hours, and would trek down to SCBU. The staff were wonderful, giving us the encouragement and confidence that quickly enabled us to do tube feeds and cuddle or bath Hannah with leads and tubes in tow. We were encouraged to make decisions, whenever possible, about Hannah's care. After the first difficult day of trying to breast-feed when Hannah was 11 days old, I asked if it was possible for Hannah and I both to be transferred to the high dependency ward to establish feeding. This was quickly put into place and helped enormously. l' 11 never forget that feeling of elation when Hannah and I were together both day and night. I couldn't stop looking at her! I found it incredibly difficult to leave Hannah at all, although as the weeks became months, I knew that I had to spend the occasional night away from the hospital."

Left: Jacob Mayo aged 17 months. This is the baby shown photographed in intensive care at the start of this chapter.

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The TOF Child "Steve and I seemed to be emotionally strong at different times, and with the help of family, friends and the medical staff, we were able to encourage one another." "On the day we left hospital I was very apprehensive as to what may lie ahead for him. I am pleased to be able to tell you, however, since bringing Craig from hospital that day, we have never looked back. I was always careful in the early days to ensure food was kept in small portions or reasonably mashed down to be on the safe side. I kept up with the communications from TOFS and took their advice; they were a great comfort through the early years. I have taught him not to `eat in a hurry' He is not under the hospital any more. The consultant has not seen him since he was nine and when we did see him it was always a happy event telling him how well Craig was." "In the parents' waiting room at the Neonatal Unit there is a photo-album of all the babies who have stayed on the unit. Most of them were of TOF babies and children who had obviously grown up and were enjoying a happy childhood. It was that album which gave me and my husband such comfort. To see those children who had been so ill like our Mark make such a remarkable recovery gave us tremendous hope."

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"I left hospital about four days after Tom was born and I spent my days at the hospital with Tom, coming home so that I was here when Ben and Sonja came home from school, then once they were in bed and we'd had a bedtime story, I returned to the hospital and stayed until about midnight. It was tiring, but I felt that it was unfair on Ben and Sonja to disrupt their lives any more." "Freddie recovered very well from his surgery and was able to come home after two weeks. He was a very tense, unhappy baby for the first five months a n d I t h i n k t h e n a t u r a l b o n d i n g process took longer for us than it would have with a 'normal' child. The trauma and worry of having a TOF baby robs you of some of the joy of becoming parents at first, but once you start to relax and live with the condition it is amazing how quickly avoiding! dealing with chokes becomes part of your routine. Freddie is now nearly three and is a wonderfully happy and physical little boy; he has only had one more stay in hospital to remove some peas he was inadvertently given at nursery. Freddie now has a brother, William. His birth enabled us to experience some of what we missed when Freddie was in special care – the pleasure of taking your child straight home from hospital and caring for him during those first few weeks – but it is strange to have a baby who at seven months could already munch his way through foods Freddie cannot manage!"


The TOF Child

Discharge from Hospital JM Beck, Consultant Paediatric Surgeon, and Penny Seymour, Neonatal Surgical Outreach Sister, The General Infirmary at Leeds. Taking your baby home from the hospital is a big step, but one that you should be looking forward to. After the trauma of the hospitalisation period you will need privacy and time to establish a routine in the comfort of your own home. Planning and preparing for discharge will help to give you the confidence you need to handle any difficulties which may arise. You will then know where to look for support, help and advice in case of problems and what to do in an emergency. Treat your baby as normally as possible; play with your child, talk to them, provide stimulation, love and security. Remember too that a little discipline and order are required in order to establish healthy routines.

Normality also entails attendance at clinics for developmental checks and routine health care. Although your TOF baby may well need some specialised care, in other respects they need exactly the same attention as any other baby.

Vaccinations It is important that your baby is protected from the common infectious diseases of childhood, so the full standard vaccination programme is essential. Despite the fears and worries periodically raised about vaccination, it is beneficial — and for TOF babies the prevention of whooping cough is particularly important.

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The TOF Child

Preparing for Discharge Babies born with OA/TOF can develop further problems after they have been discharged from hospital. It is important that you have the opportunity to learn as much as possible about these potential problems before you go home. Many of the medical professionals you meet outside the specialist unit will not be familiar with oesophageal atresia since it is a relatively rare condition. The most common problems to be aware of relate to feeding, gastrooesophageal reflux and respiratory difficulties, all of which are discussed in detail elsewhere in this book. Not all babies born with oesophageal fistula will have problems, however a significant number are affected to a lesser or greater extent. It is therefore best to be prepared. Increasing care responsibilities As the time for discharge from hospital approaches you will be encouraged to take an increasing amount of responsibility for your baby's care. If your baby is not being breast fed you will need to learn to make up and give feeds, either by bottle or tube. You will also have to measure and administer any medicines accurately and provide all the normal day to day care your baby needs. There may also be 'add on' bits of care that you will have to be familiar with, like the management of tubes and stomas. If physiotherapy, suction or oxygen are still required in hospital by the time you are thinking towards discharge, then it is likely that your baby will continue to require this at home and such treatments will need to become part of your normal routine. Medical and nursing staff will be spending time talking to you about

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your baby and what extra care, if any, you will need to learn about and continue at home. Discuss your home circumstances and the level of support available to you with hospital staff; if there are problems they are there to help. Make sure you understand When you are given explanations in hospital, do not allow professionals to get away with jargon that you don't fully comprehend. If terms are used that you don't understand, do not hesitate to ask for the advice to be repeated in simple language. Many parents report that nursing staff are far better at explaining problems and techniques in simple language than the medical or surgical staff. A number of explanatory leaflets are available, the best being produced by TOFS. Ask what leaflets the unit has, read them and discuss the contents with the staff. Be prepared The thought that you may one day need to resuscitate your baby is a very frightening one. The need very seldom arises, however it will increase your confidence enormously if you can have a demonstration of basic first aid resuscitation techniques. Many units have 'dolls' on which you can practice and videos demonstrating what to do. (A chapter discussing resuscitation is found elsewhere in this book).

The transition period On the day of discharge you will be leaving an environment where there is a team familiar with the care of babies with tracheo-oesophageal fistula / oesophageal atresia (TOF/OA) and associated problems.


The TOF Child The neonatal surgical outreach nurse, where there is one, provides an invaluable link between the hospital, the primary health care team and yourself. Your GP, health visitor and other advisors in your local primary health care team have, in all probability, never looked after a TOF baby before. The emphasis of their interest in your baby is on general well -being and child health issues, and they can offer a vast amount of experience of common childhood illnesses. These aspects are of equal importance to the care of the specific problems with TOF/OA for a TOF child. The surgical unit will contact your local healthcare team before you go home to tell them about the problems which might be anticipated and whether any medicines, equipment or special supplies will be required. Like you, they must know what action to take and who to contact if problems arise. The neonatal surgical outreach nurse will be able to provide them with additional information and assistance with regard to the TOF/OA condition, on an ongoing basis. As well as meeting members of your primary health care team, the outreach nurse can also visit you at home and ensure that your baby gets good continuity of care.

Equipment If your baby needs a feed pump, suction or oxygen at home, this will be provided and you will be taught how to use it. Health authorities vary in the arrangements they make for the supply of such equipment and with the assistance they can provide at home. Very often the surgical outreach nurse or hospital liaison visitor is the best source for this kind of information. Ensure that you know who is

responsible for the supply of equipment, how to obtain fresh supplies, replacements and repairs. When you leave hospital you should have sufficient supplies to last you at least 2-3 weeks because it takes time for fresh supplies to be organised. All these arrangements need to be planned in advance. You may find it useful to write down lists of the things you will need when you go home; you can then discuss them with staff on the ward. The more preparation you do before you go home, the less you have to worry about when you get there.

Feeding Feeding is described in the following categories: a) Oral feeding: normal feeding from the breast or by bottle. b) Tube feeding: milk is given directly into the stomach by nasogastric tube or gastrostomy, or into the small intestine by a jejunostomy tube. c) Sham feeding: for babies who have not yet undergone oesophageal anastomosis, but have been given a cervical oesophagostomy where the upper oesophagus is brought out to the surface at the neck. A gastrostomy feed is given simultaneously.

Oral feeding The normal oesophagus is a coordinated muscular tube which actively passes swallowed drink and food from the mouth to the stomach. It has mechanisms to prevent regurgitation, or reflux of stomach contents back up the oesophagus. Such co-ordinated action is not present in TOF babies because of the lack of continuity between oesophageal segments. They are therefore liable to choke, splutter and inhale feeds.

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The TOF Child These problems can lead to alarming "blue do's" (cyanotic spells) due to obstruction of breathing, and panic. As soon as the baby stops feeding and takes a breath, their colour will usually return to normal promptly. If it doesn't, tip the baby's head down o v e r y o u r knee and tap smartly between t h e s h o u l d e r b l a d e s t o dislodge anything which may be causing an obstruction. If your baby remains blue and breathing does not improve, this is an emergency and you should call or phone for help at once a n d b e gi n t he e me r ge n c y r o u t i n e which you have been taught. If your baby starts to have any problems with feeding, you should contact the surgical unit. Poor oesophageal co-ordination means that TOF babies tend to take longer to feed. They may need more breaks during their feed, and suck more slowly to allow time for the feed to reach their stomach. Smaller, more frequent feeds are often better tolerated. Some babies seem more able to coordinate sucking and swallowing if the feed is made a bit thicker. Feeding babies in an upright position encourages milk to reach the stomach more easily. Gravity will also help to prevent gastro-oesophageal reflux (a problem where the acidic stomach contents are pushed back up into the lower oesophagus, causing pain) if the baby is kept in an upright or sitting position for an hour after feeds. Difficulty with feeding will be aggravated if there is a narrowing (stricture) due to scarring at the join between the upper and lower parts of the oesophagus. Often this problem only becomes obvious when weaning to solids starts, so solids should be introduced into the diet with caution.

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Should difficulties arise, stop giving solids until you have consulted your surgeon. Introduction of solids is not recommended in any baby before three or four months of age and can be delayed much longer if the baby is growing well on milk feeds alone. (Strictures are discussed further elsewhere in this book).

Tube feeding For babies who are tube fed, it is important (with very few exceptions) to persevere with some oral feeds. In many cases, oral feeding can then be gradually increased and the tube eventually discarded, in a natural progression with no sudden changes. Tips for tube feeding include encouraging the baby to suck a dummy during the feed and placing emphasis on the social aspects of feeding by cuddling and/or looking at the baby during the feed. As the child gets older, make sure that they are included in family meal times, have the opportunity to taste and smell food and to copy nonnal eating behaviour.

Above: Anthony Pierce at 2'12 months, apparently content with his nasogastric tube.


The TOF Child Nasogastric Tube

Gastrostomy Babe

Site of anastomosis

Gastrostomy tube

If your baby is tube fed for any length of time, ask for referral to the hospital speech therapist who can devise an 'oral stimulation programme.' This will help to maintain the skills your baby will need to eat and drink normally as they grow up. A baby who does not eat through his/her mouth may not get to learn how to use their mouth normally; the exercises from the speech therapist are designed to encourage and develop muscular function and sensation in the mouth, which will facilitate the transition to normal feeding later on.

Nasogastric tubes These may be necessary in the following instances: i) babies who are unable to take adequate volumes orally. ii) babies who have severe gastrooesophageal reflux. iii) babies who have very small stomachs and need continuous feeds. iv) babies with severe oesophageal motility and/or coordination problems. v) babies who need supplementary feeds at night to improve weight gain.

If your baby is to be discharged with a nasogastric tube, you will be taught how to care for the tube, how to use it safely, how to check its position etc. Since babies pull nasogastric tubes out at the most inconvenient times, most parents quickly learn to re-pass them. For those parents who do not feel confident when their babies first go home, alternative arrangements can be made with a local community nurse or hospital.

Gastrostomy tubes If the need for tube feeding continues or is expected to continue over several months, a gastrostomy tube becomes an option. This is a tube that is positioned during a small operation, and is inserted directly into the stomach, through the stomach wall. It can be left in place for much longer than a nasogastric tube, is difficult to pull out accidentally, and is less obvious since it is hidden beneath the baby's clothing. There are a number of different tubes; if your baby needs one, your surgeon will select the most appropriate size and type.

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The TOF Child Care of Nasogastric Tubes Single-use PVC tubes are usually replaced on a weekly basis. Longer term feeding tubes made of polyurethane and silicone can he left in place for much longer periods if they do not become blocked and are not pulled out accidentally. Tubes should be used, changed and re-sterilised according to manufacturer's recommendations. The tube length and adhesive tape should he checked at least daily. Feeding Check the tube position before feeding. Its opposite end should he in the stomach, the contents of which are acidic. I f a few drops of fluid which has been aspirated from the tube are applied to blue (alkaline) litmus paper, the paper should therefore turn pink. The child should be sat up or laid at a 30 째 angle during the feed and for at least half an hour after-wards to minimise the risk of reflux and vomiting. Bolus feeds are administered either by gravity or via a suitable enteral feeding pump. The paediatric medical staff or dietician will recommend the best method of feeding your child. After use, flush the tube with 5m1 boiled, cooled water to prevent blockage and reduce the risks of infection. Vent the tube if the child is unable to burp after the feed or has abdominal discomfort or distension. This involves removing the cap or spigot from the tube, for up to half an hour, to allow air to escape. When disconnecting after feeds, attach a syringe without its plunger in place, or leave the feeding set attached; if food comes hack with the wind, it can get messy! Some children may also require venting before feeds. Re-passing tubes When re-passing nasogastric tubes always check the length carefully and mark the depth of insertion with a small piece of tape if there is no obvious mark on the tube. Try different types of adhesive tape until you .find the best for your baby and use alternate nostrils to give the other side of the nose and face a rest. Drug administration Medications should be given as suspensions, and diluted using boiled, cooled water. Any tablets must he thoroughly crushed and dissolved before administration. Flush with boiled, cooled water before and after each medication to prevent the tube blocking.

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Feeding techniques Feeds given via a nasogastric or gastrostomy tube can be given as separate feeds every 3 -4 hours, or as a slow continuous flow administered by a computerised pump. Babies with gastrooesophageal reflux or small stomachs are amongst those who m a y b e n e f i t f r o m s l o w continuous feeding. This may be given over a 24 hour period, in which case a portable pump is essential. Many babies however manage small, oral feeds during the day and have their continuous feeds over a 10-12 hour period at night. This minimises disruption to family life and encourages more noiinal patterns of eating during the day.

Jejunostomy tubes Babies with severe gastrooesophageal reflux may benefit f rom j ej unal feedi ng. Babi es who have undergone gastric t r ansposit i o n sur ger y cannot have a gastrostomy tube inserted and therefore a jejunostomy tube is the only option. The jejunum is at the top of the small intestine, below the stomach and duodenum. A tube into the jejunum can be positioned either through the nostril and stomach, via a gastrostomy or directly through the body wall to the jejunum. Jej unostomy is used much less commonly than gastrostomy or nasogastric feeding because the tube is more difficult to position and care for, and continuous pump feeding is required over 20-24 hours.


The TOF Child Care of Gastrostomy Tubes Principles of gastrostomy care remain the same whichever type of tube is used. Before cleaning the gastrostomy tube or site, and before feeding, wash your hands with soap and water. The skin around the gastrostomy tube must he kept clean and dry (use warmed saline or water) and should be examined after feeds in case of leakage. Tissue overgrowth or granuloma can he a problem around gastrostomies. Using a mild antibiotic steroid ointment (e.g. Sofradex eye ointment) on the granuloma will keep it under control. Baths are allowed; ensure the feeding port is closed and the skin around the stoma is completely dry afterwards. The end of the tube must not inadvertantly he left inside the nappy! When the stomach and stoma are fully healed, usually after 3 -4 weeks, water activities such as swimming may be resumed. Ensure the tube is well secured. Safety Check tube placement daily; blue (alkaline) litmus paper applied to fluid from the tube should turn pink due to acidic stomach contents. Check the tube length and/or position of the retention disc daily. Check the balloon weekly (if applicable; not all tubes have balloons). Ensure connectors for feeding do not stretch the gastrostomy tube. If the connectors are too large or are jammed in too tightly, the feeding port will stretch and milk will leak out. Feeding The child should be sat up or laid at a 30째 angle during the feed and for 30 minutes afterwards to reduce the risk of refluxlvomiting. Bolus feeds should be run in by gravity and should take the same time as a meal i.e. 15-20 minutes. Never use force. Continuous feeds should he administered by a suitable enteral feeding pump. The paediatric medical staff or dietician will recommend the best method of feeding your child. After use, flush the tube with 5 -10m1 boiled, cooled water to ensure 'Keeney and to keep bacteria to a minimum. Vent the tube if the child is unable to burp after the feed or has abdominal discomfort or distension. This involves removing the cap or spigot from the tube, for up to half an hour, to allow air to escape. When disconnecting after feeds, attach a syringe without its plunger in place, or leave the feeding set attached; if food comes back with the wind, it can get messy! Some children may also require venting before feeds. Drug administration Suspensions are recommended; dilute these with boiled, cooled water. Ensure that any tablets are thoroughly crushed and dissolved prior to administration. Flush with boiled, cooled water before and after each medication to prevent blocking.

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The TOF Child With a jejunostomy, the not ' tal digestive function of the stomach is bypassed, so food can be more difficult to digest. Special formula milk may therefore be needed which is easier to digest and absorb.

Sham feeding The importance of promoting normal patterns of feeding has already been mentioned. It is particularly important for babies who have not yet had an oesophageal repair and who have been given a cervical oesophagostomy. In this procedure, the blind end of the upper pouch of the oesophagus is brought out as an opening or " st oma" i n the si de of the neck, t o allow saliva to drain. A baby with such an oesophagostomy will also have a gastrostomy tube. Babies should be breast or bottle fed at the same time as giving a gastrostomy feed; the milk taken orally will of course drain out onto the neck. Any attempts to catch the fluid is usually in vain (although in larger babies and older children a colostomy bag may be useful); wrapping an absorbent towel around the baby at feed time is often the best way to soak it up. This procedure is known as "sham feeding." Giving a gastrostomy feed at the same time as a sham feed teaches the baby to associate sucking and swallowing with the feeling of a full stomach. It may seem a waste of milk, and later on of food, since it is of no nutritional value to the baby, but it is of great psychological benefit. It is preparing the way for normal eating in the future, and gives comfort and pleasure at the same time. After surgery to reconstruct the oesophagus, feeding problems are less in babies who sham fed well.

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Gastro-Oesophageal Reflux There is a valve-like mechanism at the lower end of the oesophagus which prevents return of stomach contents to the oesophagus (gastro-oesophageal reflux). The mechanism is weakly developed in most babies, hence their tendency to vomit at the slightest provocation. The mechanism increases in strength and efficiency during the first few months or years of life. Because of the abnormality of the oesophagus in TOF/OA, the tendency to gastro-oesophageal reflux can be particularly worrying and severe. It can contribute to failure to gain weight, feeding difficulties and chest infections. Treatment of reflux depends at first on posture, thickening of feeds, and drugs to improve the motility of the oesophagus and to promote emptying of the stomach. Drugs to neutralise or prevent acid secretion by the stomach can be a useful addition which also helps to prevent the discomfort associated with acid reflux.


The TOF Child There is often heated debate about the best and safest posture to counteract reflux, but there is little doubt that being kept propped up in a chair (but not slumped over) is safe, effective and allows the baby to see what is going on. When babies are lying down, reflux is reduced by lying them on their stomachs, head up, in the prone position. However, this position is not recommended for most babies because of the increased risk of cot death. If a baby has severe gastro-oesophageal reflux, the prone position may nonetheless be the safest option. If, for medical reasons, your doctor has advised you to put your baby on its stomach to sleep, then an apnoea alarm (a monitor which registers a baby's breathing and sounds an alarm if breathing stops) can give additional s e c u r i t y . I t s h o u l d b e c l e a r l y understood that any movement a baby makes will be interpreted by the monitor as breathing and therefore a baby struggling to take a breath will not necessarily trigger the alai'''. Research has shown that another good position to minimise reflux is lying on the left side, probably because it reduces the pressure on the stomach. Keeping the baby tipped head up will help to keep milk in their stomach and lessen any discomfort in the oesophagus. Raise the top of the cot or p r a m t o c r e a t e a g e n t l e s l o p e b y placing a pillow or folded blanket under the mattrress, but ensure that the slope is not so steep that the baby slips down under the blankets. Try to avoid lying your baby flat on their back after feeds. Changing nappies before feeding will make vomiting or reflux less likely than doing so when their stomach is full. For more information, refer to the chapter on strictures and reflux.

Tracheomalacia This can cause problems, but only in a minority of babies. It is a possible cause of so-called 'blue do's' sometimes seen in babies born with OA/TOF. Positioning can play a part in the management of this condition: babies with tracheomalacia can find breathing difficult when lying flat on their backs, particularly if they are crying, and may be more comfortable sat up in a baby chair. Your doctor will advise you on the safest sleeping position for your baby. Babies with tracheomalacia can have noisy breathing (stridor) when they are otherwise healthy and well. It can therefore be a good idea to visit your GP for the first time when your baby is well, so that the doctor can hear what this sounds like. You may need to explain to friends, relatives and health care professionals in the community that the noises your baby makes are quite normal for them and that they do not have an infectious condition. Babies with OA/TOF often have a very distinctive cough (referred to as 'the TOF cough') which can sound alarming to others. For more information, refer to the chapter on respiratory problems.

Medicines Most TOF babies do not require medication on discharge from the neonatal unit. Those who do may need it to control gastro-oesophageal reflux or because they have other associated anomalies (e.g. babies with renal problems may need antibiotics). If your baby is going home on medication, find out what it is, why they need it and how long for. Practise measuring it out and giving it before you leave the hospital.

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The TOF Child Giving medicines Medications Anti-reflux medication Cisapride: increases gut movements and helps the stomach to empty more quickly so that reflux is both less likely to occur and there is less material available to be refluxed back into the oesophagus. Sometimes leads to loose bowel actions, because food passes through the gut more rapidly and there is less time for foodstuffs and liquids to be absorbed. Ranitidine or Cimetidine: reduce the acidity of the stomach contents, so reducing the risk of damage to the lower oesophagus and preventing discomfort from acid reflux. Feed thickeners The following may he prescribed to keep milk in the baby's stomach and reduce vomiting: Infant Gaviscon: a powder which can he added to the feed, or given afterwards mixed with a little milk or water. Gaviscon is particularly useful for breast fed babies. Carobel: an instant thickener, which is added to the feed just before it is given to the baby. Thixo-D or Thick' n Easy: can be added just prior to feeding or mixed in advance when feeds are prepared and stored before use. Nestargel: added to recipe when feeds are made up and needs cooling to keep it smooth. Antibiotics Babies with associated renal complications may he discharged home on a low dose antibiotic. This will discourage some of the common bacteria which lead to urinary tract infections. It will not prevent all urine infections, so if the baby becomes unwell then the urine should always be checked. Feed supplements Babies who have had neonatal surgery, particularly those with feeding problems, may need extra calories in their feeds to help them grow. These are prescribed under guidance from a dietician. You will need to know how to make the feeds up before your baby goes home. Supplements are designed to give calories in the form of carbohydrates and/or fat. They come in powder or liquid form and are added to the feed when it is made up. Common examples are Maxijul, Duocal, Polycal, Liquigen and Calogen.

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Medicines are usually given to babies as a syr up fr om an o r al syringe. Many of the doses are very small and need careful measuring. Oral syringes for small doses can be difficult to obtain from the chemist at home, so take some spare ones with you from the hospital. Syringes can be sterilised either in solution or a steam steriliser. Some syringe markings fade very quickly and it may be wise to mark the correct level with (e.g.) a freezer pen as an additional guide. Although there are a few medicines which can be added to feeds, most are better given before the feed, either by syringe or in a small amount of milk. If medicines are added to complete feeds and the baby doesn't finish the bottle, you won't know how much of the medicine the baby has had. Read the instructions on the label; if medicines which are given once a day say (for example) 'give at night' then there will be a reason for this. If you are unsure what the reason is, ask before you go home. Take great care with the storage of medicines, especially if there are other children in the house. Some medicines may need to be stored in a refrigerator, others at room temperature. Remember to ask your GP for repeat prescriptions before you have run out of medicine, and allow enough time for a possible delay at the chemist in case they haven't got it in stock.


The TOF Child

Chest Infections Respiratory infections are dealt with in a separate chapter, but it is important to be aware that breathing problems can develop in TOF babies; if your baby's breathing is becoming noisier or more difficult, contact your GP or the hospital for advice. TOF babies are prone to chest infections: if they are frequent, further investigation will be necessary. Occasionally a recurrence of the original tracheo-oesophageal fistula will be detected, but more often than not no single cause is found. Contributing factors include gastrooesophageal reflux and tracheomalacia. Repeated courses of antibiotics and aggressive treatment of gastrooesophageal reflux may be needed. To help prevent chest infections, good nutrition, a healthy atmosphere and absence of overcrowding are all important. It has been shown time and again that tobacco smoke in the household is a risk; if people you know insist on smoking, keep them away from your baby and ask them not to smoke indoors.

Physiotherapy Chest physiotherapy is used to encourage drainage of secretions from the lungs in babies producing excessive secretions and/or tending to inhale or aspirate feed or saliva. It may be needed by some TOF babies at home, especially if they have a cold. The physiotherapy usually consists of various actions over the chest, while the baby is lying over your knees. It must only be used when it has been professionally demonstrated and appropriate equipment provided,

because this sort of handling can encourage regurgitation of stomach contents and aggravate the situation. Once the technique has been suitably mastered, physiotherapy should be done when the baby's breathing is moist, bubbly and noisy.

Postural drainage There are various body positions which help to drain secretions from different areas of the lung; several positions (as taught by the physiotherapist) are used at each session.

Percussion This technique loosens secretions in the chest, and is either done in a rhythmic clapping action with a small `mask' or with a cupped hand.

Vibration This is a shaking action using the hand on the chest, which aims to move secretions into the larger airways when the child exhales. During and/or after physiotherapy it is hoped that the child will cough and clear secretions. Young children may find it hard to cough out the secretions and often swallow them instead; this is not a problem. Chest physiotherapy is done before meals, usually three or four times a day. Each session should last around a quarter of an hour.

Additional Problems A number of TOFs have associated anomalies which may add to their care. Problems like the VATER syndrome, congenital heart problems, the CHARGE syndrome and others will have been described to you if your baby is affected, and any care procedures fully explained.

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The TOF Child

Follow Up Whatever happens, prolonged hospital follow up will be necessary for your baby. This follow up may be arranged at the Regional Neonatal Surgical Unit, but it is often both more convenient and efficient for routine follow up to be carried out at your District Hospital, at a special clinic attended by one of the surgeons from the Neonatal Surgical Unit together with the local paediatricians. The purpose of these follow up clinics is twofold: i) to check on your baby's progress —both the particular medical problem(s) and general growth and development, ii) to allow you to discuss problems that have arisen at home and to try to answer any questions that you have. The dosage of any medications may need adjusting to allow for the baby's growth, which is best assessed by routinely weighing the baby at each check-up under the same conditions and on the same set of scales. Arrangements for further investigations and treatment may be made; if there are feeding problems, an assessment has to be made whether these might be caused by a stricture at the anastomosis, gastrooesophageal reflux, or poor oesophageal motility. Feeding problems may be aggravated by tracheomalacia, which is often associated with breathing difficulties; signs of chronic chest problems need to be looked for and appropriately dealt with. Advice on weaning and the introduction of various foods may be given, including advice on types of food to avoid if there are problems related to oesophageal motility, stricture, or gastro-oesophageal reflux

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(the chapter 'Feeding the TOF Child' also gives further information). Remember that it is not necessary to wait for a routine follow up appointment if there is something that you are worried about. The neonatal unit or the ward where your baby was nursed, will always be happy to speak to you over the telephone, or to make special arrangements to see your baby if necessary. Keep a record of important telephone numbers next to your phone.

Finally ... Many babies have their TOF repaired, go home and thrive without requiring re-admission to hospital or any unusual treatment. It is not always possible to predict which babies will develop problems after discharge, so it is wise to be prepared. The more complicated your baby's surgery, the more likely they a r e t o have difficulties with swallowing or feeding at some stage. If your baby does have problems, be reassured that they will diminish with time. The vast majority of TOF babies grow up to be healthy adults who lead normal, active lives.


The TOF Child

Genetic Counselling for Tracheo-Oesophageal Fistula Dorothy Trump MD MRCP and Helen Firth MRCP DCH, Department of Medical Genetics, Addenbrooke' s Hospital, Cambridge. People often seek genetic counselling to find out more about a specific condition which has occurred in a member of the family. They are referred to a Clinical Genetics Centre by their General Practitioner and will be offered an appointment with either a Clinical Geneticist (a doctor) or Clinical Nurse Specialist in Genetics. During the appointment the counsellor (the doctor or nurse specialist) will often draw a family tree in order to identify any other relative who may be affected by the same condition. The process of genetic counselling seeks to answer four common questions:

What is it? Tracheo-oesophageal fistula (TOF) can occur in isolation or as part of a variety of different conditions. One UK study found that just under half the time (45%) TOF occurs in isolation as the only developmental anomaly. 1 In the remainder of instances it occurs together with other congenital anomalies. Isolated TOF (i.e. TOF without any other anomaly) is a 'one

off'occurrence. Where it occurs with other anomalies there is usually an underlying diagnosis, which falls into one of four categories: i) the commonest is an 'association' such as VACTERL and CHARGE ii) the next main group is that where there is an underlying chromosome abnormality e.g. Downs syndrome or Edward's syndrome iii) occasionally TOF is associated with exposure to an environmental agent e.g. excessive vitamin A or maternal alcohol abuse. TOF is also more common amongst twins iv) very rarely TOF occurs as part of a syndrome specified by a single gene e.g. Treacher-Collins syndrome.

Below; a family tree A geneticist will often ask for details of three generations of a family and will then draw a 'pedigree diagram' such as this to summarise the information gained. Studying the pattern of occurrence using this kind of chart helps to predict the likelihood of recurrence.

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The TOF Child The disturbance can be due to one of the situations described above – a genetic or chromosomal problem affecting this stage of development, or exposure to toxic environmental agents – but is it usually not possible to determine exactly why it happened.

Will it Happen Again? Above.. Diagnostic categories associated with TOE.

Why did it Happen? It is usually not possible to answer this question precisely. Most of the time we do not know why a TOF occurred. However, given the immense complexity of the embryological processes involved in forming a baby it is perhaps not surprising that occasionally one of these processes goes slightly awry. During the sixth and seventh week of pregnancy, the upper part of the primitive foregut divides lengthways into a digestive tract (oesophagus) and a respiratory tract (trachea).2 If this process is disturbed, a TOF may occur.

Above: Embryology ... the oesophagus and trachea develop from the primitive foregut during the 6th and 7th week of pregnancy. 50

Fortunately in the great majority of instances the answer is very reassuring and recurrence is unlikely. Isolated TOF occurs as a sporadic 'one-off' event and the chances of a recurrence is approximately 1%.3 Likewise TOFs occurring as part of an 'association' are usually sporadic and also have a low recurrence risk, in the region of 3%. In the few instances where an environmental agent has been identified, steps can be taken to avoid exposure in a subsequent pregnancy. It is therefore appropriate to be optimistic about a future pregnancy since the chance of a recurrence of TOF is low. Rarely TOF may be a feature of a rare genetic disorder. It is listed as a feature of more than 20 very rare genetic syndromes.4 Many of these follow predictable Mendelian inheritance patterns; the recurrence risk is that of the underlying disorder, which is usually high e.g. 25-50%. The geneticist will explain the inheritance pattern and recurrence risks to members of the family. In some cases a genetic test may be available (e.g. Treacher-Collins syndrome) and this would be discussed during the consultation. Specialist genetic advice should be sought, but is especially important where: i) a TOF is not isolated, but occurs in conjunction with other anomalies ii) more than one individual in the family is affected by TOF.


The TOF Child Category of individual

Isolated TOF

'Associations' (e.g. VACTERL)

Complex TOFs

1%

3%

seek individual advice

seek individual advice

seek individual advice

seek individual advice

negligible

seek individual advice

Parents with one affected child: risk to future children Individual with TOF: risk to their own children Siblings of individuals with TOF: risk to their children

negligible

Can Anything be Done to Prevent a Recurrence? With the exception of TOFs occurring as part of a rare genetic syndrome, the chance of a recurrence of TOF is low. Detailed anomaly ultrasound scanning by an experienced person can sometimes indicate a TOF antenatally. If there is oesophageal atresia the baby is unable to swallow the amniotic fluid which therefore accumulates. This is tellned `polyhydramnios.' The baby's stomach may also appear small. Absence of these signs may provide reassurance in a subsequent pregnancy.

Above: Recurrence risks for TOF.

References 1. David T. J. and O'Callaghan S. E. (1975). 'Oesophageal atresia in the South West of England.' J Med Genet 12: 1-11. 2. Moore P. 'Development of the Trachea' in 'The Developing Human Clinically Oriented Embryology' (5th edition), WB Saunders, Philadelphia: 227-29. 3. Warren J. E. K. and Carter C. 0. (1979). 'Offspring of patients with tracheo-oesophageal fistula.' J Med Genet 16: 338-40. 4. Winter R. B. M. (1996). London Dysmorphology Database, Oxford University Press.

Above: TOF Hannah Sancto with her younger sister Rebecca, a healthy baby born without the TOF condition.

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The TOF Child

Feeding the TOF Child JWL Puntis BM DM FRCP FRCPCH, Consultant Paediatric Gastroenterologist, The General Infirmary at Leeds, also Senior Lecturer in Paediatrics and Child Health, University of Leeds and Celia Shore, Clinical Nurse Specialist, Queen Elizabeth Hospital for Children, London. Feeding problems, although not always present in children born with TOF/OA, are understandably a major cause of concern to parents – who may feel that one of their basic roles has been denied by a child who will not or cannot eat. In 1988 a questionnaire survey was sent to all 230 families belonging to the Tracheo Oesophageal Fistula Support group (TOFS) requesting information about their experiences with feeding. 124 replies were received and for comparison, parents of 50 children without any kind of surgical or medical problems were also interviewed (the `control' group) using the same questionnaire. While it is possible that families of TOF children with severe feeding problems would have been more likely to answer the questionnaire than those with children who had little or no difficulties (indeed this seems to have been the case given the high proportion of children with delayed repairs / oesophagostomy), some detailed and useful information was collected which gives an idea of just how frequent feeding problems are, and how they tend to improve over the course of time. When analysing the results, the children were divided into two groups; firstly those who had their oesophagus joined up at the first operation (`early join,' 74%) and secondly – the minority – those who had an oesophagostomy before more definitive surgery at a later date (late join,' 24%).

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Breast Feeding There is no reason why TOF infants cannot be breast fed once oral feeding is permitted. In the survey, 50% of `early join' mothers and 28% of 'late join' mothers had breast fed their baby for an average period of two and a half months. This rate was a little below the percentage of all infants who are breast fed in England and Wales (about 65%, falling to 45 % by 6 weeks). Although some hospital staff may have counselled against breast feeding, there is no good reason for this in any baby who would be considered able to bottle feed, whether the surgical join-up operation was an early or late one. No mothers in the survey who breast fed reported giving up due to difficulties related specifically to a TOF repair.

Weaning and Solid Foods Feeding times should be relaxed and unhurried, with the baby held relatively upright to assist swallowing. Weaning does not need to be delayed and solids can be introduced from 3 to 4 months. This is important since it seems likely that children are particularly receptive to new tastes from around 4-6 months of age, and become more conservative if their experience remains limited. Difficulty coping with lumpy food may mean that soft puree baby foods must be given for longer than normal. When lumps are introduced, plenty of fluid should be given with meals, and the


The TOF Child older child must be reminded to chew food well and not to hurry. The social aspect of meal times is important to all of us, and eating with others has an effect of increasing food intake. The coughing and spluttering child should be allowed to recover and continue with eating rather than being made to feel excluded from the rest of the family meal.

Fibrous fruits like pineapple, oranges and blackcurrants are not suitable for weaning foods, and should be avoided.

Practical Tips on Feeding Parents need a great deal of support and advice when it comes to weaning and eating, and families should be encouraged to ask to see the dietician at each hospital visit, or a specialist nutrition nurse if one is available in their area. Questions and discussions with the GP and health visitor about feeding management should also be encouraged together with use of appropriate supporting information sheets or leaflets (see references). Parents should be encouraged to use home cooked food when changing from puree foods to more lumpy textures, since many commercial wet and dry foods contain lumps, which my cause coughing and choking

Vegetables, soft rice or pasta can be well mashed with a fork, adding pureed meat or fish. Sauce or gravy to make food moist is valuable for swallowing food trouble free. Fresh fruit may be mashed, such as banana, ripe . peaches or strawberries.

Fruits that are cooked such as apples and pears can be mixed with yoghurt or fromage frais to introduce babies and young children to more interesting and nutritious foods.

As the baby's teeth come through at about 7-8 months, encouragement with chewing may be stimulated with finger foods that are crisp or firm e.g. sweet and savoury crackers, grated cheese, well cooked carrot slices, green beans or thin strips of ham. All of these will dissolve easily once in the mouth and so reduce any coughing and choking that may occur from pieces becoming stuck. Drinks from a beaker or feeding bottle help with swallowing.

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The TOF Child

Reluctance to Feed Reluctance to feed can arise in any child who has been very ill, unable to eat, and therefore deprived of the normal oral stimulation which is part of feeding. Inflammation of the oesophagus due to reflux or stricture can also be a cause of poor feeding or feed refusal since swallowing is then painful; this may occur even when vomiting is not a prominent symptom. Most TOF children should not fall into this category. Those who cannot have their oesophagus joined up early on are given a cervical oesophagostomy and receive 'sham feeds' by mouth. Food then come out of the upper pouch of the oesophagus, which now opens onto the neck. At the same time, feeds are given directly into the stomach through a gastrostomy tube. In this way, the child learns how to suck, swallow, and to enjoy different tastes and textures until they have grown enough for further surgery which will allow them to eat food in the normal way.

Feed-Related Problems

The average age for starting solids was 4 months in the normal infants, and 6 months in the TOFs. Later on, normal infants were on average introduced to lumpy foods at 8 months and TOFs at 12 months. Whilst it is clear that even normal children may have some feed related problems, the questionnaire showed that the frequency of these had halved by weaning, and they had virtually disappeared completely by the age of one year. In contrast, TOF children were five or six times as likely to be considered slow with feeds or to experience coughing and choking, and twice as likely to vomit during feed or refuse to take milk altogether. This situation had not changed very much by the time solid food was being introduced, but did seem to improve considerably over a longer period of time. Of 88 TOF children at one year of age, only 18 (20%) were free of all feed related symptoms, but amongst 21 children who had been followed up for s e v e n y e a r s , 1 2 ( 5 7 % ) w e r e completely symptom free.

The following graph shows how often parents reported feed related symptoms with two or more milk feeds each day.

Once children in 'late join' had undergone further surgery and closure of oesophagostomy they had a similar frequency of feeding difficulties to `early join' children.

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The TOF Child

Troublesome Foods Although it is clear from this and other studies that feeding problems generally do get better with time, some TOFs may continue even in adult life to associate swallowing problems with certain types of food which they then avoid. This is probably because there is still some incoordination in the oesophagus. In the survey the most frequently identified "problem" foods were meat (37% of children), apple (23%), bread (23%), oranges (14%) and raw vegetables (12%). These are foods which take longer to grind up when chewing and tend to be swallowed as larger lumps. In fact, a further 38 problem foods were listed, but often only one item was mentioned by any one family. Making sure that food is well chewed seems to be the message here, together with the acknowledgement that it is often impossible to predict whether or not particular foods will cause difficulties in any one individual.

Feeding Difficulties There are a number of reasons why TOF children have feeding difficulties. Normally, food is propelled through the oesophagus by a wave of muscle contraction which sweeps it along down to the stomach. In TOF children, this process is never completely normal (even in the child without symptoms). Coughing or choking occurs when incoordination of the oesophageal muscle means that food or fluid stays in the oesophagus and spills back into the trachea. Sometimes on a radiograph, fluid can

be seen to go up and down the oesophagus like a yo-yo. Less commonly, there may be a narrowing (stricture) at the point in the oesophagus where the surgeon has joined the two ends together, which simply doesn't open up to let food through. This is usually caused by a natural process of scarring as the wound heals, and is treated using dilatations (stretching). Gastrooesophageal reflux is another cause of feeding difficulties; both of these problems are discussed in a separate chapter. Sometimes food — or occasionally an unusual object swallowed by a young child, often termed a 'foreign body' — may get stuck in the I— oesophagus and cause a blockage (obstruction). This can be either 'functional' (due to incoordination of the oesophageal muscle, so that food is not propelled down to the stomach) or `mechanical' (due to a stricture i.e. a physical narrowing of the oesophagus due to scar tissue such that solids cannot pass through). Whatever is involved in the blockage, the obstruction it causes may mean that swallowing even saliva from the mouth is impossible. If the child becomes very distressed or the blockage fails to clear within an hour or two despite taking sips of water, hospital assessment will be necessary. You should telephone your local paediatric unit and arrange to go to the ward. This kind of problem is more common in the early years after surgery and rare beyond ten years of age.

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The TOF Child

Oesophageal Function Normal: wave of peristalsis moves food down the oesophagus

Oesophageal incoordination: muscular activity is abnormal

Stricture: narrowing prevents food passing through the oesophagus

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The TOF Child

Summary From the survey it appeared that most parents felt they had not been warned about feeding difficulties likely to be encountered at home, or given advice regarding diet and weaning. Some parents stated that feeding difficulties dominated their lives and many expressed feelings of isolation and helplessness.

Further Reading 1. Tracheo Oesophageal Fistula – TOF's Guide to Weaning Foods. Leaflet by Celia Shore, sponsored by Milupa and available from the Queen Elizabeth Hospital, London E2 8PS. ISBN 1 898081 15 8. 2. "What's for Dinner Today, Mum?" A short guide to weaning. Leaflet available from the Department of Health, PO Box 410, Wetherby, LS23 7LN. 3. Puntis JWL, Ritson DG, Holden CE, Buick RG. Growth and feeding problems after r epai r of oesophageal at r esia. Arch Dis Child 1990; 65: 84-88 (this is the TOFS survey). 4. Smith IJ, Beck J. Mechanical feeding difficulties after primary repair of oesophageal atresia. Acta Paediatr Scand 1985; 74: 237-9. 5. Orringer MB, Kirsh MM, Sloan H. Long-term esophageal function following repair of esophageal atresia. Ann Surg 1977; 186: 436-43. 6. Dellert SF, Hyams JS, Treem WR, Geertsma MA. Feeding resistance and gastroesophageal reflux in infancy. J Paediat Gastroent Nutr 1993; 17: 66-71.

In children who have been joined up early on, swallowing difficulties should be anticipated – although some seem to have no problems at all. Experienced nursing staff, dieticians, speech therapists and occupational therapists may be able to give useful advice. Much practical and emotional support for parents struggling with feeding difficulties has come from other parents within support groups such as TOFS, many of whom have seen their children get over their problems and grow to be strong and healthy.

Quotations from TOF s, their families and friends and medical professionals "Jacob is now 17 months old and won't eat or drink anything. We try and be positive that things will improve, but feel we need more help on the psychological difficulties of re-learning swallowing and not retching and panicking every time food goes into the mouth. We have had to fight hard for the support we do receive, which is hard with all your other worries."

Above; James Wharfe at 1 year, who had a gastrostomy tube until 27 months old. At 14 years old, he is said to eat like a horse!

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The TOF Child "The pressure on TOF families is huge. When you tube feed it's such a performance wherever you go. My husband used to go fishing and I was so desperate one day to get out of the house I took the stand and fed her out of the car window in a field. The sickness and mess was always a problem. You were always tense hoping she wouldn't bring any food back and at other children's parties you had to explain to people without being boring. Those curled up bibs were a godsend."

"Breast feeding was a very difficult task. After surgery Catherine refused the breast and would fight whenever she came in contact. I expressed and tried to put her to the breast for 10 minutes at every feed. At about 4 weeks I discovered nipple shields and so she fed from me happily for the first time. After a hernia repair at 3 months while still half asleep she fed without the nipple shield! It was a wonderful moment. Catherine was breast fed for 17112 months. I feel that breast feeding her was my greatest ever achievement."

"On buying him his first ice-cream cornet he turned it upside down as he was more interested in the wafer as he hadn't been allowed it before."

"I have always been told he will eat one day, but as the months pass you do start to wonder if people are telling you the truth. Well last month Matthew actually took some yoghurt in his mouth and swallowed it. I just couldn't believe it, I don't know how to explain it. He still takes small amounts but it is a start and I can actually see a small hole at the end of the tunnel."

"We used to lie to strangers about his age – he only weighed 12 lbs aged one. a photographer said how alert he was for a 10 week old (he was 10 months at the time but I didn't explain.) He gulped some fillet steak which he had taken from someone else's plate age 6. The surgeon said there was enough meat for a pie stacked in his gullet. I was very embarrassed, it seemed as though I'd been feeding him giant fillet steaks." "Our lasting memory of the postoperative period has to be the sham feeding, and the look of horror on the face of a workman, who was artexing our ceilings. He came to ask me a question as I was feeding Alexander and the vision of me standing with a syringe of milk in one hand and a bottle in the other along with the squelching noise coming from Alexander's neck region, was all too much for him, he made a very fast exit." "But we have to admit all the hard work of sham feeding is worth every second, just to see Alexander tucking into his meals with no problems is a pleasure."

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"I had no idea what was in store when I started giving him solid food. It took hours to get anything into him; even scrambled egg had to go through a sieve, and often enough he brought it all back up again. At one stage, when he was a little older, I used to count out, individually, ten Rice Krispies, which (if I was lucky) he could eat, without milk. He used to look at the food the other children had, and say hopefully 'tiny bit?' (having heard me say he could try a tiny bit on a few occasions). He longed to eat crisps, but could never keep them down. It w a s w o n d e rf u l w he n w e f ou n d h e could cope with Skips. His brother and sister naughtily taught him to make a sicky noise and say "been sick," which had me rushing up with a cloth a few times, to find it wasn't true!"


The TOF Child "I nearly burst with love and pride when we first took him to a Beefeater Restaurant (he was 3) and he managed a Mister Men meal of soup, fish-stars, potato-letters and baked beans, followed by clown sundae ice-cream, without getting stuck once."

"Rachelle' s school friends don't tend to invite her home for tea or to parties because their parents are terrified of her getting something stuck and not being able to cope. This is very unfair on Rachelle. We have had to stop going out to restaurants so often and can't just pop into McDonald' s because Rachelle can't cope with the food there." "We know that Olivia feels different at mealtimes, at nursery, birthday parties and family gatherings. She looks nervous and uncomfortable. As she watches what others are doing you can tell she wants to join in by eating normally but doesn't feel she can or know how." "Our son has been extremely lucky in that he has never had any problems following the repair of his OA at birth. He was breast fed until 11 months; went on to solids at four months and eats whatever we eat. In the beginning I would never let anyone else feed him I would not have those worries now."

Above: Freddie Clamp-Gray enjoying his 2nd birthday party with the help of Wotsits, thin crust pizza and potato salad. "One amusing thing that Philippa sometimes does when she's stuck is to bounce around on her hopper. I don't know if it helps but she at least feels as if she's doing something." "She is used to drinking at mealtimes – between mouthfuls and before she starts eating – and even close relatives cannot help themselves commenting th at the f luid wil l f ill her up. She ignores this and so do we, because this together with chewing food very extensively has enabled her to eat more or less "normally." If she feels unable to cope with something in her mouth, she will remove it – say grape skins or a tough piece of meat."

"Laura's school provides supervised lunch times but they call me in if she has a bad choke. I asked them to normalise the situation as much as possible which they have done. I don't want Laura to feel too different from the other children."

"Eleanor has been attending nursery school for a few hours a week since she was 19 months old. She stayed for lunch from 21 months old and we ensured the nursery nurses were fore-warned about Eleanor's occasional problems of getting food stuck. Also that they were not to put any pressure on her to eat (I could top her up with a gastrostomy feed when I got her home). We feel sure that Eleanor was encouraged with her eating by sitting with and watching other children at mealtimes."

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The TOF Child "He started on solid foods (a very, very sloppy consistency) at five months old. I failed at all attempts to introduce any second stage foods and eventually gave up trying until I felt he was more able. He ate 'mush' until he was quite old. He disliked intensely any commercial baby foods and I always gave him ordinary food blended and sieved. His general health improved during the summer of 1996 and I requested to visit a speech therapist after reading an article on a child with an eating disorder. Through my own efforts we visited a wonderful lady who specialised in feeding difficulties. This visit began the road to recovery. Kris was introduced to 'bite and dissolve' finger foods which he took to instantly. I was advised never to introduce lumps into food but just to make the consistency firmer. Over the next few months I followed her advice to the letter and Kris made rapid progress. He is now 23 months old and eats an almost normal diet. I do not however give him foods that I feel may cause a problem. I spend more time preparing Kris's food. He eats all cooked veg and fruit, but I avoid giving him all foods of a solid consistency such as raw veg, apples and foods in a natural skin such as sausages and oranges. Meat he has shredded to a crumb-like state."

"Harriet's brother is four years old. He is wonderful with Harriet and asks if she can eat things before he gives her anything. He has accepted her problems more easily than anyone." "Sometimes when I have things like lamb chops or chewy meat I feel a lump in my throat, so to get rid of it I push with my thumb to help the food go down." "Having a TOF child is a full time job. My son Matthew is 2 and is still tube fed overnight and we have just started introducing food to him. A meal time for Matthew takes over an hour to complete. I can't go to work as I can't leave him with anybody." "...one of the hardest things is when you see other children eating in, say, a MacDonald's – they don't know how lucky they are. Matthew is the happiest, most contented child I know. He never cries, whines or complains even though he has probably had more operations than most people would have in a lifetime."

"Sarah says she feels different at school dinner time because she has two drinks instead of one, and other children say it's not fair. She tells them it's because she gets `stucks' ." "Whenever Sarah attends a party with her friends I tell the parents her problem but without trying to alarm them. School have been good, but sometimes I feel if she hasn't got something stuck for some time, they forget to give her extra drinks. I have to go and remind them."

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Above: Leslie Brown aged 11 months. Leslie took some 16 months to get to the stage of attempting to eat finger food; at 11 months she had a jejunostomy tube.


The TOF Child

Strictures and Reflux Mark D Stringer MS FRCS FRCP, Consultant Paediatric Surgeon, Leeds.

Both of these are quite common in infants born with oesophageal atresia with or without a tracheo-oesophageal fistula, but only a minority will require open surgery to correct the problems.

What is a Stricture? A stricture is the commonest problem requiring surgical treatment after a TOF repair, occurring in about one third of babies, particularly in the first few months after surgery. It is a narrowing in the oesophagus, usually due to scarring at the join (the anastomosis) between the upper and lower parts of the oesophagus, in which case it is called an anastomotic stricture. Occasionally, a stricture develops lower down in the oesophagus and in this area it is usually due to gastro-oesophageal reflux. Very rarely, children with TOF/ OA are actually born with a stricture in the lower oesophagus; this is termed congenital stricture. Anastomotic strictures are more likely in certain infants, in particular, those where the surgical join in the oesophagus has been particularly difficult because of the wide gap between the ends of the oesophagus. If the anastomosis is under tension, the blood supply to the join site is

Symptoms of Stricture Typically, symptoms develop within a few weeks or months after the baby's oesophagus has been joined up. The baby may be slower to feed or may choke and splutter during the feed. This is not a 'one-off' episode, but happens with each feed. A stricture may become apparent for the first time when the baby is tried with solid food. Advice should be sought from the surgical unit. Not all swallowing problems are due to strictures. We know that abnormal contractions of the oesophagus (called dysmotility) and difficulties with learning to feed are important causes of feeding problems in some TOF children (see Chapter 'Feeding the TOF Child'). In older children with a stricture, there may be difficulties swallowing lumpy food but it is important to remember that TOF children often have difficulties in this area anyway. Types of Stricture

impaired and it will tend to heal as a tougher and more fibrous scar. Some of the old-fashioned stitch materials like silk were more likely to cause strictures. Strictures are also believed to be worsened by gastro-oesophageal reflux; acid from the stomach damages the delicate join in the oesophagus and encourages more scarring.

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The TOF Child If a child has previously coped well with a certain consistency of solid food and then starts to have problems with swallowing, this suggests a stricture.

Dealing with obstructions Occasionally, a piece of solid food may actually get stuck in your child's oesophagus (called a bolus obstruction). This may be vomited back or remain stuck and cause distress. Once the child has calmed down, see if they are dribbling their saliva. If they are, then there is a complete blockage and you will need to seek expert medical attention. If the child is not dribbling, try and get them to sip some water since food materials will often dissolve and pass through. If the child cannot tolerate liquids within an hour or two then you will need expert advice.

A food bolus obstruction can happen in an oesophagus with only a minor narrowing and typically occurs with unchewed lumps of meat, apple, sausages, etc. However, a food bolus obstruction can also indicate a stricture that needs dilatation. Very occasionally, a stricture comes to light if a child swallows an unusual object (known as a foreign body) such as a coin which then gets stuck.

Diagnosis of Stricture A stricture can be confirmed using either of two techniques:

Radiography The child is given a drink of barium or some other safe liquid which shows up white on a radiograph. This is done in the radiography department where the passage of the dye (called a contrast material) is watched in motion as it goes down the oesophagus. The use of X-rays in this way is called fluoroscopy. A stricture may be seen. Alternatively, there may be minimal narrowing in the oesophagus in which case the feeding difficulty has another cause such as oesophageal dysmotility.

Endoscopy The other way to diagnose a stricture is to look down the oesophagus with a telescope (called oesophagoscopy), a procedure which requires a short Left: Anastomotic stricture: contrast material (e.g. barium) is seen outlining the o e s o p h a g u s d o w n to th e s t o m a c h (t h e white area at the bottom). An anastomotic stricture can be seen about a third of the way down this view. The white stripes passing from top left diagonally downwards a re th e rib s; th e thicker ha nd passing them at right angles in the top left is the spine.

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The TOF Child general anaesthetic and is necessary in cases of persistent food bolus obstruction since the obstructing material can be removed with the aid of the endoscope. Grasping forceps can be passed through the endoscope and the material withdrawn with the scope, or the food can be dissolved with squirts of water, or occasionally, the food bolus can be simply pushed on down the oesophagus into the stomach.

Treatment of Stricture A stricture can be successfully treated by stretching it up (called a dilatation). This is done under a general anaesthetic but can be carried out in several ways. Most commonly, the surgeon passes a telescope (called an endoscope or oesophagoscope) down through the mouth to the level of the stricture. Then a fine plastic rod is passed through the stricture. A series of gradually larger rods are then passed through until the stricture has been sufficiently dilated.

An alternative method is for an Xray doctor (radiologist) to pass a thin flexible wire (known as a guide-wire) through the stricture whilst checking its position with a radiograph. Once

Above:

Foreign body in the oesophagus. The patient has swallowed dye (contrast material) which has flowed around the foreign body to outline the oesophagus. The foreign body (the circular, white area) is lodged above an oesophageal stricture.

the wire is safely through the stricture and into the stomach, a thin hollow plastic tube is passed over the wire and down into the oesophagus. This tube has a balloon on the end which can be gently inflated when it lies across the stricture. As the balloon is blown up, the stricture is dilated (called balloon dilatation).

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The TOF Child When performed by experts both methods are safe and effective and relieve the symptoms. The child is able to feed within a few hours and is often able to go home the same day or soon after. A chest radiograph may be performed to check that there is no sign of a complication (see below). A very small number of strictures keep coming back despite repeated dilatations. In these circumstances any gastrooesophageal reflux must be treated since the presence of stomach acid in the oesophagus may be aggravating the situation. Occasionally, further operative surgery may be needed. Some surgeons try injecting the stricture scar tissue with steroids which have an anti-inflammatory effect. This injection can be carried out with a special needle through the endoscope. The idea is that when the stricture is then dilated it might heal with less scarring and therefore be less tight. Other options include actually cutting out the stricture and rejoining the oesophagus or considering a major operation to replace most of the oesophagus. Such operations include removing most of the oesophagus and using a piece of large bowel to bridge the gap between the healthy upper oesophagus and the stomach (colonic interposition) or bringing up the stomach through the chest and joining it to the upper oesophagus in the neck (gastric transposition). (See chapter on Oesophageal Substitution Procedures).

Complications of Stricture Dilatation Stretching a stricture can lead to complications but these are rare when the procedure is performed by experienced personnel. The most worrying complication is

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splitting the oesophagus which causes it to leak (called an oesophageal perforation). This is most often recognised by seeing a pocket of air outside the oesophagus on the chest radiograph or if the child becomes unwell after the dilatation. An oesophageal perforation can be dangerous and requires prompt treatment with antibiotics, intravenous fluids and sometimes, a tube put into the chest to drain away any infected fluid. Oesophageal perforations are rare and this kind of treatment would allow most to heal up. Only rarely would an operation be required for this complication. Any child born with a cardiac (heart) abnormality ought to receive antibiotics before the dilatation to minimise any chance of an infection settling in the heart. Anastomotic strictures usually get better after one or two dilatations but occasionally, several stretches are necessary. If the stricture keeps coming back, it might be because there is a particularly dense scar at the oesophageal join, it may be because gastrooesophageal reflux is making the stricture worse, or it may be because there is a congenital stricture. These problems all require separate solutions which may involve further surgery.

What is Reflux? Gastro-oesophageal reflux (GOR) is the involuntary passage of gastric contents into the oesophagus. With GOR, as the stomach empties into the bowel (the duodenum), part of its contents are also squeezed back into the oesophagus. Since the stomach cont ent s ar e aci di c, i r r it ati ng aci d passes into the oesophagus. The lower oesophagus has several features that normally prevent GOR


The TOF Child causing problems. There is a sort of valve in the lower oesophagus made up of a high pressure area, a sharp angle between the oesophagus and stomach and a pursing of the lining of the oesophagus at its junction with the stomach. This valvelike mechanism is not st r on gl y d e vel op ed i n nor ma l babies and explains their tendency to vomit. This tendency reduces during the first few months or years of life as the antireflux mechanisms in the oesophagus get stronger. In TOF babies, GOR is even more common. This is partly because the oesophagus has not developed normally and partly because repair of the oesophageal atresia often pulls the junction between the oesophagus and stomach upwards, weakening the antireflux barrier. TOF babies can therefore vomit more easily than normal babies. In time the tendency to GOR lessens, but in some children with repaired oesophageal atresia, GOR causes major problems. GOR is more likely to be a problem if there has been a wide gap between the oesophageal ends and the repair has been particularly tight. Antireflux mechanisms

Symptoms of Reflux Remember that all healthy babies have a tendency to GOR which improves with time, so the question really is whether GOR is causing a problem in your baby – rather than whether GOR is occurring at all. Reflux causes frequent vomiting after feeds. This is not the small mouthfuls of vomit (`possets') seen in all babies, but the vomiting of large amounts of the feed. This can happen immediately after a feed or occur repeatedly right up until the next feed. If GOR is severe, the baby has great difficulty gaining weight because he/ she is unable to absorb all of the feed. Other problems may also occur. The oesophagus may become sore from the acid (the adult equivalent which is heartburn) leading to irritability and poor feeding in the child. In some cases, bleeding from the oesophagus causes anaemia or signs of blood in the vomit (haematemesis). Anastomotic strictures can be made worse, as mentioned previously. Rarely, reflux can happen so quickly that it leads to the baby inhaling vomit, leading to a chest infection or difficulty with breathing. In severe cases, the baby may temporarily stop breathing (called 'apnoea'). Most TOF babies have mild reflux ( i .e. a t endency t o vomi t i ng af t er feeds) which gets better by itself or with medicines but a few have severe reflux which needs intensive treatment with several medicines or even an operation.

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The TOF Child

Diagnosis of Reflux

pH Study

Listening to the history often gives the doctor vital clues as to whether GOR is causing problems. Sometimes, it is difficult to tell whether a TOF baby's feeding problems are due to GOR or a stricture or some other problem. There are several ways to diagnose GOR.

This involves passing a fine tube through your child's nose down into the lower oesophagus. A radiograph is sometimes necessary to check the position of the tube. The tip of the tube has a small sensor which measures the surrounding acidity (or pH) which is recorded by a small computer. The pH study usually runs for 24 hours, during

Radiography The commonest method is to get the child to swallow some dye (contrast material) in the radiography department. Normally, the stomach contracts and empties the dye into the first part of the bowel (the duodenum). The dye is prevented from passing upwards into the oesophagus by the valve-like mechanism mentioned above. If the child has gastro-oesophageal reflux, as the stomach contracts to empty, the dye is also seen travelling up the oesophagus. The severity of the reflux can be assessed by how much dye passes upwards and whether it just enters the lower oesophagus or passes right up to the throat.

which the child can eat and drink and live a fairly normal life within the constraints of the equipment (as carried by the boy shown here). Any antireflux medicines must be stopped before the test to avoid giving a false reading. Normal children have a little acid in the lower oesophagus for brief periods only (less than 5-10% of the time) but children with significant GOR have acid present for much longer periods. Left: Gastro-oesophageal reflux: contrast material (white) is seen passing up the oesophagus from the stomach (the round, solid-looking area on the right), as indicated by the open arrows. The slightly cloud-like accumulations of contrast material below and to the left of the stomach represent stomach contents which have passed on beyond the stomach into the duodenum and small intestine.

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The TOF Child Endoscopy

Milk scans

Persistent gastro-oesophageal reflux of acid causes the lining of the lower oesophagus to become inflamed (called oesophagitis) and this redness and soreness can be seen by endoscopy (oesophagoscopy).

These are used in some centres. This technique is also known as gastrooesophageal scintigraphy and involves the baby taking a small amount of radioactive material by mouth, followed by a milk feed; the baby is then scanned by a special camera which detects the distribution of the radioactive marker. The test does not require sedation and the radiation dose is less than that involved in performing a barium meal. The presence of radioactivity in the oesophagus indicates GOR but there is none of the anatomic detail seen with a barium swallow which is the more popular investigation.

Treatment of Reflux

Above: Oesophagoscopy: normal lower oesophagus, viewed through an endoscope.

This depends on the severity of the reflux and how much trouble it is causing your child. Mild GOR, which is probably present in all TOF infants (and many otherwise healthy babies), tends to improve spontaneously with age and often gets a little better when the baby is able to wean on to more solid food. Simple measures that are helpful include changing the position of your child (posturing) and, in babies, thickening the milk.

Posturing

Above: Oesophagoscopy: endoscopic view of lower oesophagus, with oesophagitis.

There has been some argument about the best and safest posture to counteract reflux. GOR tends to be worse when lying flat and therefore a gentle raise of the head of baby's cot can be useful. This can be done by putting a pillow or folded blanket under the mattress to create a gentle head-up slope. Never attempt to let your baby sleep directly on a pillow which could be dangerous. During the day, keeping your baby propped up in a chair (but not slumped over) can help prevent reflux. Changing the nappy before feeds makes vomiting

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The TOF Child less likely than doing so when his/her tummy is full.

Milk feed thickeners There are many varieties of milk thickeners. These are some of the commoner ones: Carobel, Nestargel Made from Carob seed flour. Added just before a feed to thicken it instantly. Thixo-D, Thick and Easy Made from maize starch. Can be added just before a feed or mixed in advance when feeds are prepared and stored. Gaviscon A powder which can be added to infant formulas or given after a breast feed. Feeding your baby with slightly smaller volumes of milk given at more frequent intervals may also be helpful. If your child has bad reflux with frequent vomiting, oesophagitis, recurrent stricturing or poor weight gain, then treatment with antireflux medicines or antireflux surgery will be necessary.

Antireflux medicines Antireflux medicines work in different ways. In general, they either reduce the severity of the reflux by improving the downward movement (i.e. motility) of the oesophagus and stomach, or by reducing the acidity of the stomach so that the reflux is less damaging to the oesophageal lining. These are the names of some commonly used drugs: Motility drugs Cisapride (Prepulsid ®) and domperidone (Motilium ®) act by strengthening the antireflux valve at the bottom of the oesophagus and speeding up stomach emptying. There has been a lot of concern about cisapride in premature babies and this has caused a 'knock-on' anxiety concerning the use of this

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drug in GOR generally. Most clinicians are still using the drug since it can be very helpful; there are, however, various precautions to be taken, which you must discuss with your doctor. Acid lowering drugs Cimetidine (Tagamet ®) and ranitidine (Zantac ®) are the drugs most frequently used to lower the stomach's acidity. Omeprazole (Losec ®) is a more powerful acid-lowering drug. Other medicines Gaviscon contains antacids and tends to float on top of the feed, thickening it. Any medicine can have side effects but these are not known to be common with these drugs. Occasionally, a child may get diarrhoea with cisapride, develop unusual movements or a rash – in which case you must stop the medicine and seek medical advice. Cisapride must not be given together with certain other medicines such as erythromycin (an antibiotic) or fluconazole (sometimes used to treat thrush) because harmful effects have been reported in this situation. Any unusual reaction to a medicine should always be reported to your doctor and the drug should be stopped at least temporarily. Medicines are usually given to babies as a syrup from a syringe which allows you to measure small doses. You should be given a supply of syringes from the hospital as small syringes are n o t a l w a y s e a s y t o g e t f r o m t h e chemist. Most of these medicines are best given before feeds. However, Infant Gaviscon is supplied as sachets which need to be mixed with the feed or, in breast fed infants, mixed with water and given after the feed. R e me mb e r t o s t o r e t h e me d i c i n e s safely out of reach of other children (some need to be kept in a fridge).


The TOF Child Antireflux medication often needs to be continued for many months and can then be gradually removed when the reflux has improved. In some children, reflux is persistent but mild, causing occasional heartburn or discomfort but never severe enough to need surgery. In these patients, antireflux medicines need to be used occasionally or only when they have symptoms. Sometimes, infants and children do not get better with the m e d i c i n e s o r t h e y h a v e m a j o r problems with reflux such as repeated stricturing, chest infections from overspill of gastro-oesophageal reflux

Nissen Fundoplication Fundus of stomach Sling around oesophagus

into the lungs, persistent severe oesophagitis or inadequate weight gain. In these children antireflux surgery has to be considered.

Antireflux Surgery The usual antireflux operation is known as the Nissen fundoplication. There are others, for example the Thal fundoplication – the choice of antireflux operation partly depends on what your surgeon believes gives the best results –but for TOF children the Nissen operation is usually the one selected. The Nissen fundoplication is a fairly big operation. Most paediatric surgeons do the operation through a cut in the upper part of the tummy but some surgeons are evaluating whether doing the procedure through multiple telescopes (`keyhole surgery') has any advantages.

Procedure Oesophagus Opening in diaphragm for oesophagus

Duodenum

Stomach

Th e fu n dus of th e s t o mac h is wrapped around the lower oesophagus, creatin a valve to prevent refl ux Fundoplication

Opening in diaphragm tightened

In the Nissen fundoplication, the lower oesophagus is freed up and the top part of the stomach (known as the `fundus') is wrapped around the lower oesophagus to make a valve at the junction of the oesophagus and stomach. The hole in the diaphragm through which the oesophagus passes is also tightened. With an experienced surgeon, the operation can be done safely with few serious risks.

Use of gastrostomy tube Sometimes a feeding gastrostomy is inserted at the same time to provide supplementary feeding afterwards. Gastrostomy feeding may be given as a continuous overnight feed with a pump or as separate volumes (boluses) during the day or a combination of both, depending on the needs of your child (see the section on gastrostomy feeding in the chapter on Discharge from Hospital).

Gastrostomy tube

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The TOF Child Post-operative considerations Creating a valve at the lower end of the oesophagus prevents reflux of gastric acid, thereby allowing any oesophagitis to heal. Vomiting is abolished and stricturing improves. T h e operation is usually very successful but has some disadvantages. Firstly, the child may not be able to burp and can therefore be more `windy' (called gas bloat); this often improves after a few months and if there is a gastrostomy tube in place, you will be taught how to wind your child through this. Secondly, the child may have some retching instead of vomiting; again this often disappears after a few weeks or months. Because part of the stomach is used to create the antireflux valve, the stomach volume is a little smaller to begin with and smaller, frequent meals are needed. In a crisis, your child may learn to vomit past the valve but if your child is poorly and keeps trying to vomit, it is best to open the gastrostomy tube to relieve him/her. If your child does not have a gastrostomy

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tube and is unwell with repeated retching, it may be necessary to bring him/her to hospital to have a nasogastric tube passed to relieve the discomfort. It is especially important to seek medical attention if your child has bad tummy pain and retching since this could mean a blockage in the bowel due to an internal scar (adhesions) from previous surgery. Rarely, a fundoplication causes intermittent diarrhoea or sweaty episodes (`dumping') after a feed or, if the valve is too tight, swallowing difficulties occur. Repeat surgery Nissen fundoplication may stop working properly in some cases after a few months or years because the valve weakens and then some of the symptoms of GOR return. In this case, antireflux medicines may be effective but if the reflux is severe, the operation needs to be repeated. A 're-do' Nissen fundoplication means undoing what is left of the old stomach wrap and doing the operation again.


The TOF Child

Respiratory Problems in TOFs by Una M MacFadyen, Consultant Paediatrician, Stirling Royal Infirmary.

The Respiratory System The respiratory system starts at the nose and extends through the trachea, bronchi and bronchioles to tiny air sacs (alveoli) in the lungs, where oxygen passes from the air to the blood. Every tissue in the body needs oxygen for it to work properly; oxygen carried in the blood is pumped around the body by the heart and delivered to the tissues. Because of the vital importance of respiration, the body has natural defence systems in the respiratory tract to protect it from damage and to help it to recover from illness or injury. The lining cells of the respiratory tract are uniquely designed to do different jobs. For example, the hair and cells lining the nose filter out dust and other particles in inspired air. Some of these cells (goblet cells) also produce mucus (a specialised, sticky fluid) that will mop up anything that might irritate the airway; mucus also helps to keep the air warm and moist, Ciliated Cells and Goblet Cells

The Lower Respiratory Tract

Trachea Bronchi

Bronchiole Right lung

protecting the lower airway from getting too dry. Other cells (`ciliated cells') have tiny arm-like projections (`cilia') which wave continuously in a to-and-fro motion. Collectively, these cilia act like a microscopic conveyor belt which carries particles trapped in the mucus to the pharynx where both the mucus and its trapped impurities can either be swallowed or coughed out. Action of Cilia

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The TOF Child Fur t her down t he air way, cel l s lining the trachea do a similar job in clearing even smaller impurities in the air. Here the cilia have to carry the mucous coating upwards (and therefore against gravity) to the pharynx; this is known as the `mucociliary escalator.'

During infections (e.g. coughs and colds) mucus production is increased to speed the removal of bacteria. The airway also becomes more sensitive meaning that accumulations of mucus or irritant substances are quickly coughed out. Reflex coughing also occurs if something goes down the wrong way on swallowing.

What is different in the TOF? In the commonest type of TOF/OA there is a blind ending upper oesophagus (the atresia) and a connection between the lower end of the oesophagus and the trachea (the fistula). Other forms of TOF have different connections but the effect on the tissues of the oesophagus and trachea are the same: i) the muscle of the oesophagus is interrupted at the atresia and even after the repair there will be an area of the

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oesophagus that does not transmit swallowing action (peristalsis) as smoothly as normal. ii) the supporting tissue of the trachea called cartilage (the gristle) is not fully formed at the site of the fistula. The cartilage forms arches around the front of the trachea to give it support and to hold it open all the time. Without this cartilage the trachea is softer than it should be. If there is a long section without cartilage this can cause floppiness of the trachea called tracheomalacia. iii) the air filter cells (goblet cells and ciliated cells) are replaced at the site of the fistula by less specialised cells (squamous cells) that are less efficient at keeping the airway protected. After a TOF repair a deficiency is therefore present in the mucous coating and the conveyor belt system. In t he bab y bor n wi t h T O F/ O A there is an abnormality of the structure of both the respiratory tract and the oesophagus. The trachea sustains a major surgical injury in the first few days, yet in almost all cases recovers well so that there are no long term serious problems by the time the TOF child has grown up. Respiratory consequences of the differences in the oesophagus The problems with swallowing in a TOF child can have secondary effects on the respiratory tract. In the immediate newborn period before the oesophagus is repaired, saliva or any milk that they try to swallow will spill over into the trachea causing coughing and choking. After the TOF repair this "spill over" can still happen if the repair site in the oesophagus narrows (oesophageal stenosis) so that swallowed liquids get held up in the oesophagus. Some babies with TOF develop problems with gastro-


The TOF Child oesophageal reflux, when the lower end of the oesophagus has a tendency to fill up with stomach contents after a meal. This sometimes allows aspiration (the passage of material into the respiratory tract) to happen quite a long time after drinking or eating. A few such babies tend to wheeze when there is reflux into the oesophagus without aspirating, so different kinds of tests and treatments may be needed to find out what is actually happening before treatment can be given.

growth of the normal section of the trachea and the tracheal cartilage is firmer, the deficit is easier to cope with. In addition, many normal babies tend to reflux during the first year, so any problem related to reflux will therefore show at its worst. The repair site occupies a large part of the young baby's oesophagus so any section that is narrow or has poor activity has a major effect on function.

Respiratory consequences of the differences in the trachea Most people hardly notice that they have any mucus to clear and just automatically clear their throat while the cells lining the trachea do the rest of the job. In a TOF child, the gap in the normal protective lining cells (at the site of the fistula) can be enough to make the clearance of mucus less efficient and/or the mucus drier than normal. The child may therefore have to cough hard to clear this mucus.

Most babies who have had a TOF repair do not have major difficulties coping with tracheomalacia and it becomes less and less significant as they get older. There are however a small number who have serious problems because the trachea actually collapses so that no air can pass through it. In its most severe form this complication causes problems which have been referred to as 'near death episodes,'when the baby seems to be choking and unable to breathe. Because these are rare, many doctors have never witnessed one of these episodes. It helps enormously if parents are able to describe what happens in as much detail as possible, since the child is often perfectly well by the time they get to hospital or are seen by a doctor.

The `TOF cough' results from the need to clear this dry mucus by coughing through a trachea with a slightly floppy section that makes the flow of air less smooth during the cough. Anything that increases mucus production will make the TOF cough worse, e.g. colds, liquids or food getting into the airways (aspiration) or asthma. When are problems at their worst? The problems with the respiratory tract following TOF repair tend to be at their worst in the first two years. This may be because the trachea of normal infants contains immature cartilage which is quite soft. The area of absent cartilage from the TOF trachea has a much greater effect when surrounded by a softer cartilage framework; when there has been more

Tracheomalacia

The factors causing the airway collapse can sometimes help to explain the time of the attacks and the way in which the child is affected: Airway collapse when breathing in When we breathe in, we suck air into our lungs through the airways. The lungs are filled with air like a bellows as the chest wall moves out and the diaphragm muscles move down. The walls of the airways outside the chest tend to be pulled in during this

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The TOF Child

Effects of Tracheomalacia

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The TOF Child action. Normal people feel this when they try to take extra large breaths through the nose and feel a 'drag' on the windpipe. If the trachea is floppy this 'drag' draws the walls of the trachea together; without the normal supporting cartilage the trachea may close off completely. This is like suffocation and is very frightening for the baby (and anyone who witnesses it). The baby goes blue and may lose consciousness or have a brief convulsion. This is obviously very dramatic, nevertheless the baby relaxes as he/she becomes unconscious which allows the trachea to open up again; breathing is then at once effective. If the baby is still conscious with their mouth open, gently pulling the tongue forward may help as a first aid measure to open the airway. If however the baby is unconscious and not breathing, blowing gently into the airway by mouth to nose-and-mouth respiration is the correct course of action.

Airway collapse when breathing out If the trachea is floppy in the lower part (within the chest) then its walls will be pulled open during breathing in (inspiration). Breathing out (expiration) is usually simply a relaxation from the effort of breathing in and causes no difficulty. The only times when a physical effort is made to breathe out are when crying or coughing hard. Babies with severe intra-thoracic tracheomalacia may experience tracheal collapse during these moments. As above, they can go blue and lose consciousness, at which point they relax and the airway opens up again. If a quick recovery does not occur, the airway can be gently blown open with mouth to nose-and-mouth breathing.

Diagnosis The infant with severe tracheomalacia usually becomes symptomatic around the age of 4-6 months when there may be an exaggeration of the TOF cough, excessive wheezing or cyanosis (blue attacks) during feeding. In extreme cases, the infant may experience acute lifethreatening episodes. For a doctor, the story of a baby going blue with hard crying may suggest common breath holding attacks. However, these tend to occur in older toddlers who are either angry or very upset, and happen at the end of a big breath in or out; the 'near death episodes' usually happen in the midst of a normal crying spell. Observers who can observe and describe these kind of details about the episodes can help greatly in making a correct diagnosis. In addition to problems due to the pressure changes of breathing efforts, a soft trachea can also be squashed from the outside. This may occur when a narrowed oesophagus causes a hold up, so that the upper oesophagus fills up and stretches so much that it pushes against the neighbouring trachea. Without a strong enough cartilage framework to keep it open, the trachea can become closed off until the oesophagus empties – either by a vomit or by the contents passing through the narrowed region. The same situation can arise if the lower oesophagus fills up with refluxed stomach content. The latter episodes can be hard to explain as they ma y n o t s e e m t o b e l i n ke d t o a n y reason for a breathing problem, but again the more detail observed the better.

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The TOF Child

Tracheal Compression by Oesophagus

Oesophagus Left lung

Diaphragm Stomach

Distension above stenosis

Swallow fills out oesophagus above stenosis Wall of trachea squashed

Distension following reflux

Reflux distending oesophagus

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Wall of trachea squashed

Tests for tracheomalacia There are a number of different tests that can be done to help with diagnosis of tracheomalacia, but since there is often more than one problem present it can be difficult to work out which is the most important for the baby, and then to decide on the best treatment. Most babies have some floppiness in the trachea after a TOF repair, but the majority are not troubled by it and any problems will improve with age. It is therefore not enough simply to look for floppiness, especially when a problem in the oesophagus may actually be the origin of the symptoms. Tracheomalacia also needs to be distinguished from gastro-oesophageal reflux, which can present in a similar fashion. Radiography The diagnosis of tracheomalacia can be suspected on a lateral (side-on) radiograph of the neck during inspiration and expiration, which will show a collapsing trachea. A barium meal study is often useful to check for any oesophageal malfunction. Bronchoscopy Bronchoscopy (looking into the airway via an endoscope) is the most reliable method of reaching a diagnosis. The area of collapse is classically 2-3 cm above the bifurcation (splitting) of the trachea, where the tracheooesophageal fistula entered the trachea. Respiratory function tests Tests of the baby's breathing can measure how much work the baby has to do in breathing in and out, but cannot give information about the length of the trachea which is abnormal. When the extent of the problem has been ascertained, treatment options can be discussed and any actions planned.


The TOF Child Treatment of tracheomalacia Surgery for tracheomalacia aims to give the trachea extra support.

Tracheopexy This operation involves placing stitches to attach the wall of the trachea to the back of the breast bone (the sternum). These sutures anchor the trachea and make it more difficult for the walls of the lower (intrathoracic) trachea to collapse together. The procedure can help if the tracheomalacia is mostly in the lower trachea and the baby has problems during expiration. Aortopexy The aorta is a major artery which lies in front of the trachea. In aortopexy, the aorta is displaced forwards by suturing it to the back of t h e sternum (breast bone); this maintains the trachea in the open position. The procedure acts at the precise site of the collapse in the lower trachea and is instantaneously effective in relieving the obstructive symptoms. Which of these operations is used depends on the site of floppiness and what the surgeon thinks will work best for the individual baby.

Tracheostomy Both of the above operations work best for intrathoracic tracheomalacia that is short enough to be supported by a few stitches. If the floppy part of the airway stretches down into the smaller airways (bronchi) it may not be possible to give the wall of the trachea support f rom t he out side. In such cases, a tracheostomy tube may be inserted to ensure that the baby has a safe airway. This involves making an opening in the front wall of the tr achea in the neck, and placing a little tube through the opening to hold the trachea open all the time. In severe cases of bronchomalacia (where the floppiness

stretches beyond the trachea into the bronchi) it may be necessary to apply CPAP (Continuous Positive Airway Pressure — a constant flow of air or oxygen at a pressure that gives extra support to the airway from the inside) or the modification known as BIPAP. Very few TOF children have this type of problem and most who do have a good chance of improving greatly with treatment and age.

Infections There is no evidence that TOF children have more respiratory infections than other children, but they may have more trouble coughing enough to clear the airways of the extra mucus during normal infections. Most childhood chest infections are caused by viruses. The body fights these by producing specific antibodies which endow the child with resistance to future attacks by the same virus. This defence system is called the immune system; TOF children have a normal immune system and make antibodies in the normal way. Viruses are not killed by antibiotics; some viruses irritate the airways and cause them to become swollen and tight. Problems arise with TOFs"normal' chest infections for two reasons: i) the impaired clearing of airway secretions can allow mucus to settle in the lungs. Bacteria (germs) can gather in these local accumulations of mucus and cause a more serious infection to develop. This is often called a 'superinfection' because it is on top of the first infection — not because there is anything very special about the germ involved. Bacteria are killed by antibiotics, so if there are signs that a virus infection has not cleared naturally then antibiotics are recommended. Often antibiotics are given right from the start 77


The TOF Child of respiratory infections in young TOFs because they are likely to have difficulty coughing up phlegm. As the child gets older he/she may not need so many antibiotics. Taking antibiotics does not stop the body's own defences from fighting the virus and the child will build up immunity in the normal way. Most bacterial infections respond to the common antibiotics, but occasionally extra courses or wider acting (`broad-spectrum') antibiotics are required. This especially applies if an area of the lung has abnormally small airways, as with bronchomalacia or bronchiectasis (where the walls of small airways are weakened and stretched so that infection Constriction of the Airway in Respiratory Disorders Normal mucus

Normal small airway bronchiole)

Lining Muscle in wall

Tight (constricted) e.g. mild asthma Muscle tightens temporarily

Swollen with mucus (infection) Swelling

Tight and swollen with mucus (severe asthma or asthma plus virus infection)

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collects in tiny sacs deep in the lungs). In such cases chest physiotherapy helps to clear secretions from the lungs and antibiotics may be prescribed for an extended period (weeks or months) until the lungs are completely clear of signs of persisting infected phlegm. ii) the airways of TOF children may be more sensitive to viruses causing swelling and tightening, making it hard to move air in and out. The effect is the same as is seen in asthma and responds to the same type of treatment.

Prevention of sensitive or tight airways (`wheeze') When the airways narrow from swelling or tightening, the child feels that he/she cannot breathe properly. This is very frightening and can be dangerous if so little air moves that the amount of oxygen reaching the blood is reduced. There are several ways of preventing and/or treating this situation. i) protect the lungs as far as possible by having the child immunised against whooping cough, Hib (Haemophilus influenzae), measles and, if advised by your doctor, against influenza and pneumococcus. ii) avoid situations that cause the problem. This may be easier said than done, but for example if you know the child is sensitive to animals then it is wise to avoid getting a furry pet. iii) one of the commonest causes of wheezing in young children (and also of more troublesome respiratory infections and ear infections) is being in an atmosphere where people smoke (passive smoking). Avoiding smoking in the home where chesty children live is therefore important.


The TOF Child Treatment of sensitive or tight airways ('wheeze') i) keep calm and know what to do to help the child. The muscles which tighten around the airways react to fear and anxiety; children are very sensitive to fear in adults, so it helps if adults know how to deal with breathlessness and can give the child confidence. Learn the basic treatment steps, what to give when and how to get help when needed, and you will be helping your child. ii) medications: 'drug' is the technical name for medication i.e. the word does not mean the same as the addictive drugs you read about in the papers. These fall into two main categories: Relievers (bronchodilators) These work by making the tight muscles around the airway relax and are most effective when inhaled directly into the lungs. They act quickly which `relieves' both the breathlessness and the fear that accompanies it. The effect wears off in four hours or less, so treatment may need repeating frequently. There are various inhaler methods which allow different ages of child to breathe in the medication: puffers: metered dose inhalers. spacers: demand less co-ordination to

use but are bulky. dry powder inhalers: where the child

sucks the dose into the lungs. nebulisers: convert the drug into a fine mist which is administered through a mouthpiece or mask. Several pharmaceutical companies manufacture relievers, so there are a number of different names for the same kind of drug (Ventolin, Bricanyl, Aerolin). Most in the UK come in a blue container, so this is the one to use when sudden tightness occurs. The technique for use of relievers

should be explained by a doctor or nurse who can make sure that the treatment plan makes sense both to the child and parents, and that when and how to get emergency help is fully understood. Other less common relievers which act in slightly different ways may be recommended for children with more troublesome asthma symptoms. These have a longer lasting action; examples include salmeterol (Serevent) and theophylline (Uniphyllin). The wheeze of some very young babies is more due to swelling than tightening of the airway muscles and so they may not respond to the usual bronchodilators. Such babies may be treated with ipratropium (Atrovent). Preventers (prophylaxis) Because the airways are sensitive even when they are not tight, it is often preferable to use a regular treatment which helps them to withstand whatever irritates them. This both reduces the number of breathless attacks and makes reliever treatment more effective. Preventers are administered in the same ways as the relievers. There are two main types – sodium cromoglycate (Intal, in a red and white pack) and inhaled steroids (usually in a brown or orange pack, e.g. Pulmicort, Becotide, Aerobec). Although the steroid preventers are related to the

Inhaler ...follow the instructions provided by your doctor or as given by the literature with the inhaler

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The TOF Child strong steroids that are taken by mouth for other conditions, the dose is so small when breathed into the lungs that they are much safer than oral steroids. They are not at all like the body building steroids that some athletes take against medical advice. If the airways become very swollen, the response to relievers may not be adequate. The child may then need a short course of stronger steroids (e.g. prednisolone), taken by mouth, to reverse this potentially dangerous problem. Your doctor will advise when this is required. When used in this short term manner, the risks of steroids are far less than the risks of being unable to breathe.

Outlook in the Longer Term TOFS children, on average, have more respiratory symptoms than other children. This is most marked in the first nine years, however in most cases they can be just as fit and active as their friends of the same age, albeit perhaps needing some medication to help. As they grow older any symptoms often improve greatly, although specialised breathing tests may still show differences from normal. Perhaps with the exception of activities depending on above average lung function or those where exposure to airway irritants is common, there should be no restrictions on what a TOF can do for work or for leisure.

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Quotations from TOF s, their families and friends and medical professionals "However much you convince yourself that it doesn't bother you, it is actually very difficult to cope with the stares and comments you receive when your child barks away in John Lewis." "Henry has his amazing TOF cough which always causes much attention. It causes even more of a stir when he does it in conjunction with picking up a discarded cigarette butt in the park!" Below: Craig Palmer, 10 years.


The TOF Child "It wasn't until I had sat in a GP's waiting room with Eleanor who gave a "TOF cough" did I realise the true intolerance of other people! The bark is there, particularly on those days when she has a cold and chestiness. People will always stare or comment, and there was a time waiting to be seen in a Casualty Department that some people actually moved away from us! Even today, it is the most common statement made to her – "Oh, you have got a bad cough"– even among people who are aware of her condition!" "Strangers told us we shouldn't take out a baby with such a bad cough –this became very irritating and gave insight into the impact of well-meaning but ignorant people who assume all kinds of things." "Sometimes my brother will wake up during the night coughing and spluttering. When I was young this used to scare me because I thought he was choking but now I understand the problem it's OK." "The rest of the family have dealt with the TOF condition very well – maybe they fuss a little too much over asthma, colds and the TOF cough which we get used to and learn to ignore the stares and comments of complete strangers."

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The TOF Child

Growth in TOF Children PAT Chetcuti DM FRCP FRCPCH, Consultant Paediatrician, The General Infirmary at Leeds

Introduction

Growth Charts

Parents of TOF children are sometimes concerned about their child's growth. For these children, milk — the source of energy promoting growth — is unable to pass through to the stomach and small bowel at birth. Even following corrective surgery, complications may be present which threaten the supply of nutrition, mostly in the first two to three years of life.

Growth charts are available for children, based on measurements in large numbers of normal children of different ages. Charts exist for males and females and consist of different centile lines. The 50th centile line is the average line. The majority of children have heights between the 97th centile and the 3rd centile.

This chapter considers noll _ ial growth, factors which influence growth and growth as it specifically relates to oesophageal atresia.

Normal Growth The most important influence on a child's height and their future height as an adult is parental height. Generally, tall parents have tall children and small parents have small children. However, a tall and a short parent may have a child who is tall, average or short, and occasionally the height of grandparents has a bigger influence on a child's stature. Despite these obvious observations, many genetically small children are referred to the medical services for assessment.

In assessing growth in children, a single measurement is of limited value. Several measurements over a period of time — preferably at least a year — will determine the rate of growth, known as the height velocity. The height velocity might show that (for example) a short child is growing normally or a tall child has stopped growing. height (cm) 180 160

97th centile 50th centile

140 120

Right: Representation of a growth chart, showing the different centile lines. Different charts are used for boys and girls; usually a series of measurements are taken of height to determine the 'growth velocity.' (Illustration based on the Buckler Tanner growth chart, © Castlemead Publications) 82

100

3rd centile

80 60 —age (years) 0 2 4 6 8 10 12 14 16 18


The TOF Child Crucial Periods in Growth

Growth Hormone Deficiency

Growth is at its fastest in the first 20 weeks of foetal life. Any major health events in the early weeks of the pregnancy can have a significant effect on the process of growth. Growth in the first 2 years of life is also rapid, and – like foetal growth – is predominantly dependant on adequate nutrition. Whilst growth remains important throughout childhood, the final important phase of rapid growth occurs in adolescence; prior to this growth spurt, the growth rate tends to slow down. Prepubertal growth occurs mainly in the limbs whereas pubertal growth occurs mainly in the trunk. Growth hormone is important in influencing growth in older children, and the sex hormones have a role in the pubertal growth spurt.

This is rare, occurring in 1 in 4,000 children, and can occur at any age. Children with this problem either fail to grow or grow poorly despite a d e q u a t e n u t r i t i o n . C a r e f u l investigation reveals that their growth hormone levels are low. It is very important to make this diagnosis because these children respond well to growth hormone replacement.

Factors Affecting Growth in Children Nutrition Lack of nutrition, to the foetus or in the first year of life, for whatever reason, may result in stunting of growth –possibly with lifelong consequences.

Chronic Illness Rare chronic disorders affecting the bowels, kidneys, heart and lungs can affect growth. This is the result of a c o mb i n a t i o n o f d e cr ea s e d ca l o r i e intake and the increased energy requirements that children have in coping with these illnesses.

Syndromes and Chromosomal Abnormalities There are a few rare conditions which may be inherited or occur by chance in which children are born with a number of different abnormalities and despite good nutrition do not grow normally.

Children who grow poorly for the other reasons stated above, including inadequate nutrition, have normal growth hormone levels and therefore do not respond to extra doses of growth hormone.

Growth of TOF Children When assessing growth in a TOF child, one must consider two factors; size at birth and subsequent growth. Some infants with oesophageal atresia are small at birth. The reasons for this are unclear because in foetal life the nutritional supply comes from the placenta. It may be that those with poor growth at birth have other major problems such as heart problems which have affected their growth. In a review of over 300 children and adults born with oesophageal atresia the majority had grown normally. The graphs on the next page illustrate the distribution of height measurements on the centiles. The findings are very encouraging. While there are slightly more in the lower growth centiles, p r o v i d i n g e v i d e n c e t h a t g r o w t h pr obl ems and poor wei ght gai n ar e mor e prevalent in chil dr en under 5 years of age, the vast majority have a final adult height which is normal. In practical terms, parents may be relentlessly pursued about their child's undernourished appearance by well-

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The TOF Child meaning relatives, friends and health professionals inexperienced in managing children born with oesophageal atresia. This can cause unnecessary distress and anxiety which may rebound on the children themselves, affecting morale. In the longer term however the figures suggest that the majority experience `catch up' weight gain to achieve a normal final adult height.

Inadequate Nutrition If there are significant problems with feeding, such as solids impacting the gullet (oesophageal obstructions) or vomiting, then the nutritional intake may be reduced. This is most likely to be a problem in the first years of life. With very careful management however – stretching of narrowed areas, and medical and sometimes surgical treatment of gastro-oesophageal reflux – nutritional depletion can be minimised. Some TOF children may be given oral concentrated nutritional diets or supplements to boost calorie intake; very occasionally a gastrostomy (a tube inserted directly into the stomach) is needed to maintain nutrition.

Above:

Percentage distribution of all TOF patients for height centiles: there are slightly more patients in the lower growth centiles.

Respiratory Problems For the first few years of life after surgical repair, it is common for TOF children to have a persistent cough and/or recurrent minor chest infections. Rarely, the chest infections may be more severe and infants may be chronically breathless. In this rare situation, the extra energy used to breathe out can result in poor weight gain because the dietary intake is not adequate to allow for this.

Other Associated Problems Half of all children born with oesophageal atresia have other significant abnormalities. The majority of these abnormalities are not major. However some children may have

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Above:

Percentage distribution of adult TOFs for height centiles: the distribution is normal, indicating that any growth problems in early life have been overcome. abnormalities that might affect growth, particularly children with heart problems, chromosomal abnormalities or syndromes. Growth holinone deficiency is not associated with oesophageal atresia, therefore growth hormone treatment is not beneficial in short children or children with poor growth velocities.


The TOF Child

Who to talk to if you are worried about your child's growth Parents with concerns about their children's growth should make contact with the surgeon and centre where the initial surgery took place to discuss their worries. For parents moving around the UK, all Medical Schools and Teaching Hospital Centres in the country will have a Paediatric Surgical Department with experience in oesophageal atresia and its

complications. Advice and referral to these can be obtained directly from a General Practitioner. Another important source of help is the Liaison Surgical Nurse Specialist. These are very experienced Senior Nurses attached to most Paediatric Surgical Units who often visit and get involved with families at home. Remember that even though problems may seem very difficult at the time, the longer tern' outcome in teiins of growth is very good.

Quotations from TOF s, their families and friends and medical professionals "I wonder whether the TOF condition has affected Thomas' growth, particularly in the early years when his diet was limited, or should I say hampered, by the condition. Did it actually put him off eating quantity of food?" "I feel different when all my friends are doing things I can't do and when people call me small!' "We would like Chris to gain a little more weight and be a little taller. It only worries us when he gets upset because he says he is the smallest in his class." "Craig is eleven now. He has never had problems with eating, he eats everything – he has never needed a dilation. The only reminder is a `TOF bark' when he has a cough. He only has the occasional chest infection, no more than any 'normal' child. He has lots of stamina, he plays football, golf and swims very well. His weight is average for his age, he's a super boy!"

Above: James Wharfe, 14 years, who had a gastrostomy tube until the age of 27 months. After various set-hacks James now enjoys an active life and is reported to eat `like a horse' although remaining slim.

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The TOF Child

The TOF Family Dr Glenys Parkinson, 0 T PhD (Developmental Psychology) is a Chartered Clinical Psychologist, working at the Brookside Family Consultation Clinic in Cambridge, where she often uses play therapy to help children with emotional psychological problems. She also has 15 years' experience in the paediatrics department of Addenbrookes Hospital, Cambridge, where she works closely with the play specialists who prepare children for medical procedures. This chapter looks at the way the TOF condition affects the family. Letters and questionnaires from many families have documented the experiences of TOF children, siblings, parents, grandparents and friends. Extracts from these communications have been grouped to illustrate the key points, with an accompanying narrative.

Initial Anxieties It is devastating for parents to realise that their soon to be born or born child has a serious problem. When the news comes that surgery is required, their joy is turned into anxieties... how can such a small baby be operated on? "Waiting for Freddie to come out of surgery was dreadful. We were both exhausted but unable to rest; we had only just got to grips with the basic condition as described by the surgeon and until the operation was over we would not know the extent of the problems. We had barely held our baby and now his life was in someone else's hands." "I wondered how a baby with such enormous physical problems could survive. Feeding is such a fundamental requirement that it necessarily affects relationships in ways which are impossible to know."

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The message that comes through from those who contributed to this book is that parents do cope — not without cost — but they manage to give their child as normal a life as possible. It isn't easy. "I think my sister wishes the family would sometimes realise the extra work load she has and the worry she often feels. Because Matty looks and acts like any normal child, people don't realise the extra work and stress a TOF child can give." Life with a TOF child is seldom easy with demands being made upon all three generations of the family. Daily life and routines are never the same again. But as time goes on, consideration turns to the wider view; what about babysitters, nursery school, children's parties?

Getting Help and Support A child in a wheelchair gives other people a notion of what to do; a child with no obvious sign of difficulty may be overlooked by others, not taken seriously enough, or taken so seriously noone is willing to take responsibility. "Over the years-we have come up against problems getting people to understand TOF. Parents of school friends don't understand and think you are a bit neurotic and overprotective. The school have been quite good but


The TOF Child there is still a lack of knowledge. That has always been an obstacle – trying to get the message through. They often take it quite lightly and think you are fussing! This makes one quite angry and a little anxious should your son get into difficulties when having tea somewhere. Then, if you over explain they get frightened of feeding him!" The focus on the child is a two fold problem: it is very difficult to avoid being overprotective, and such a focus tends to make siblings feel this child is special and is 'worth' more than they are. "As a baby my family were frightened of handling Emma and wouldn't babysit. However, they call her their `miracle' and have a closer relationship with her than they might have done were she not a TOF. Grandparents still panic when she goes into hospital and one set cannot face visiting." There is an understandable terror of dealing with a child who may choke. Coping with this means acquiring the skil ls and f eel ing pr otect ed while doing so, in order that the anxiety may diminish. Parents normally get the opportunity to learn the necessar y skills during the period when their child is in hospital. Since every child is different these skills are fine -tuned over a period of time and it is easy to forget that others have not had the benefit of this supportive environment when the child has been discharged. However, it is important for all sorts of reasons that others are able to feed and handle the TOF child. It helps to take the focus off the affected child if an aunt, uncle or grandparent can feed all the children, not just the `nolinal' ones. It also means that if parents fall ill or are otherwise engaged, there is someone who can be called in to help.

Once the parents have gained confidence in dealing with the baby, it might be helpful to have a grandparent or baby-sitter watch for some time; then deal with the baby themselves with mother or father standing by. Then with the parent in the kitchen, in the garden, outside the gate ... "Grandparents spoil him and treat him as though he were made of china but as time goes on they are getting better" Eventually the carer may have the confidence to deal with the child themselves, having done so in protected circumstances. It is very important that both the carer and the parents feel confident that the baby is in safe hands. Communication and time can reap enormous rewards in this respect. "The family dealt very well with his condition. We always asked lots of questions so everyone has a good understanding and was involved in bringing about Louis being well."

Spreading the Load The pendulum can swing the other way; t he par ent s feel such guilt at having produced a baby with a TOF t h at t he y w a n t t o ' ma ke u p f o r i t . ' Parents' job is to protect their young; when they feel they haven't done so of course they want to compensate for it. However, in reality parents also need time to be on their own and should not hope to devote every moment to the child, nor to take responsibility for every component of their care. To attempt to do these things does not help anyone. It would be useful if consultants could tell parents that it is NOT their fault – nobody could have prevented it. The best way to bring a TOF child up is to treat him or her the same as their sibs whenever possible. This will

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The TOF Child not be feasible all the time because of the risks of choking, the anxiety over having someone else look after the child, the problems of diet, weight, growth etc. Nonetheless, there are ways of sharing these burdens. If possible the parent should find a doctor, dietician and health visitor they have confidence in. The dietician can advise on appropriate food for growth, the doctor or health visitor on development. Parents do not have to carry the whole of the burden themselves; they carry the most onerous part – the anxiety – already. Let someone else, with the necessary knowledge, do the monitoring. When there is the need to seek immediate medical attention, it may be arranged with the consultant beforehand to go straight to the ward where the child has been treated (if this is local) to be seen by the consultant or senior registrar; the nurses should also know what to do. If these kind of arrangements are not made for you, it is well worth asking; a note from the consultant confirming that this permission has been granted may also be beneficial. If the specialists who treated your child in the early weeks and months are not local, alternative arrangements must be made; this book may also be of assistance.

What to Tell the Child? When is the best time to tell the TOF child about their difficulties? It has been found to be useful to do so as soon as possible. From a very early stage the child should be told in words he or she can understand what the difficulties are. Most children are aware of their condition from an early age and their first attempts as language may lead them to describe the feeling if something is stuck in their oesophagus. Teinis such as "Got a stuck," or "Got stuck" followed by "Got stuck gone" are examples.

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Explanations from the parent or carer can be very simple at first, for example "When you eat something, you have a tube leading from your mouth to your tummy. The food you eat makes you grow. Your tube is not as wide as other children's, so you need to drink to make the food soft." A demonstration can be made with some plastic tubing and wet sand, which has difficulty passing down the tube. Water allows it to pass freely. Young children need to see something 'concrete' rather than just a picture or photograph – which will only mean something to them when they are rather older. "There have been some lighter moments over the years however. It was very comical hearing my two year old son coming out with words straight from a medical dictionary. He often asked our friends and visitors if they had an oesophagus."

When young the child can feel very alone. The presence of the parents' visit can make all the difference and provides the basis for a secure future. "When I was younger I used to get ill in the night and cough. My mother, who is a light sleeper, used to hear me and come and see me. It used to make me feel very close to my mum. Now I don't cough so much but I still remember the feeling of closeness."

As the child's understanding grows, the information can be elaborated on. Always explain things truthfully, however in simple terms. That way information can he expanded and parents don't have to say: "It wasn't quite how we told you then." Some families find that compiling a scrapbook with photographs starting from birth, including some of the child


The TOF Child in hospital and then at home, accompanied by simple captions is beneficial. Looking at these may be painful for parents, but children are often fascinated by a book about them and can accept what has happened in a very matter-of-fact way.

The Child's Viewpoint Children soon learn how best to get their parents' attention and a TOF child may use his/her condition to test boundaries at home. If a child can manoeuvre parents into doing things he wants, he feels he is stronger than they are. It may bring satisfaction in the short term, but in the longer term it makes the child feel unsafe because underneath it all, he knows how frightened he can be. If he is stronger than his parents, who can take care of him?

"Jake already understands that feeding is an issue that causes frustration and concern and he can play on the stress we feel as parents." Young children with illnesses or disabilities often think they are being punished for something. An explanation of their condition helps this, but one or two play therapy sessions with an experienced play therapist can also assist. Children express things with play they don't have words for; thoughts come to the surface and can be expressed non-verbally in play. This gives the therapist – who is seen as authority – the opportunity to say: "Some children think that because they have something different about them, they have done something wrong. I am telling you that this is not so. It is very unfair you have trouble with your throat and lots of other children don't, but it is not your fault."

"I recall enormous stress around feed times as a child but didn't think much of it. Now, as an adult, I can recognise an underlying sense that I was at some level 'at fault' for any problems in the family. Later in life, to understand how the anxiety around my condition must have affected the family, and to recognise that it was not my fault, is an enormous relief"

Siblings The child is not the only one who may feel guilty. Siblings often resent a newcomer in the family, or they resent the time taken for a child with a disability. Some even wish they were the TOF child. These feelings are absolutely normal and children should be encouraged to express such feelings, hard though it is for parents. As with the TOF child, the siblings should have explanations as soon as possible. "I'm told that when my mother was

pregnant with Simon my favourite game was using her as a trampoline, and I've always felt a sense of guilt that somehow this was connected to his being a TOF, it was only when I really understood what TOF is that I no longer felt this guilt." "Looking back I appreciate how hard my mother tried to spend time with us older kids and to visit Simon in hospital. However, I was only two when he was born, and one of my earliest memories is resenting the fact that my mother was never at home at bedtime. I didn't really get to know Simon until he was permanently at home, before that he was just something covered in wires. In fact I only enjoyed going to visit him because of the hospital play room."

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The TOF Child Play Therapy Dr Glenys Parkinson uses Projective Play Therapy which was developed by Margaret Lowenfeld. The child uses the available toys as they wish, to express thoughts and feelings which may not have been given words. The therapist reflects back what the child is doing and may make general comments such as "lots of children may think ..." This is a non-intrusive form of therapy; the relationship between the child and therapist is not worked with — only the child's thoughts and feelings, as expressed through play. Children love coming for this work and look forward to it.

In sand tray work, the child is given a free choice from a selection of toys.

Which children benefit? Children exhibiting the following behaviours may benefit from play therapy:  Behavioural difficulties  Being sad over a long period  Short temper  Aggression  Worries over illness  Withdrawal, inability to make friends Making friends is possibly the best prognostic sign and is therefore important to take note of. Friends are a valuable source of support and without them, a child may feel very isolated.

Wet or dry sand can be used: the

therapist reflects on what the child is doing, always working with the child.

Referrals GPs will normally refer to a psychiatrist at the local Child and Family Centre, who usually works with other colleagues such as Clinical Psychologists, Speech Therapists or Occupational Therapists. These work together as a team, and the most appropriate person to see the child and family will be decided on together. After the first consultation, the family may be seen together, or the child may be seen individually. Not all clinics offer play therapy.

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Mosaics are a little different; whereas sand tray toys have their own identity, mosaics can be given meaning(s) from the child's thought's and feelings. The variously coloured shapes can be assembled into something which is either concrete or abstract. The child's thoughts and feelings about the mosaic are explored afterwards.


The TOF Child On the other hand they may also feel protective, and worry about their sibling with TOF.

"I get worried when she goes into hospital. I don't like her being away. I like playing with the hospital toys and visiting the nurses. I know sometimes she has to go into hospital but I don't like her upset." "I love him so much because I waited so long for my brother to come home from hospital. He was so small when I saw him born, but I couldn't cuddle him with his tubes. I like him, he's nice, he's special to me. He's my brother" "Emma and I are very close (mother) and we both try not to overprotect but probably fail. We do try and encourage her to be physically active to build her strength and become adventurous. Adam, her five-year-old brother, has a good relationship with Emma, helped by the fact she has always brightened and smiled even on her worst days in hospital when he comes to visit her." It is quite possible for these contrasting feelings to go together, one being experienced one day, the other another – or even both together.

"I couldn't help feeling a bit jealous of all t he ti me my parents spent wit h Colin. However, I also remember feeling a great affection for Colin and a sense of protectiveness towards him. One memory which sticks in my mind is trying to feed Colin a packet of crisps, which he found very difficult to eat. I sat with him patiently for what seemed like hours, giving him tiny pieces of crisp alternated with a drink. He almost managed it, and I was so disappointed when he ' got stuck' as we used to call it. People who didn't know about his TOF would get alarmed when he started to choke and cough,

but for me it was just a part of life. I forgot that other children didn't have to worry about what they ate. It is not until now, looking back, that I realise how incredibly brave and courageous Colin was, to struggle through he hard times and keep going." Having a brother or sister with TOF brings its problems, however many seem to have gained in some way from the experience.

"It was very traumatic and stressful at times but we feel that we have learnt a great deal from our younger brother's stay in hospital." "Tim was almost four-and-a-half years old when Matt was born. We explained as best we could why Matt was still in hospital and how we would have to gastrostomy tube feed him. Tim took this all very well, and so when I was allowed home from Aberdare Hospital we took Tim with us to see Matt for the first time (my husband had been to see M a t t se v er a l t i m es b ut T i m a nd I hadn't seen him). Tim wasn't allowed into the Special Baby Care Unit and so he had to be lifted up to look through the glass in the door. The nurse lifted Matt from the incubator and held the open ended gastrostomy tube aloft. Tim looked lovingly at his little brother and then, in a loud excited voice, said, "I can see his stomach pump!" "

Friends The support of friends can be a huge help to the TOF child, their siblings and parents. For parents, in particular, friends can be an important safety valve, giving them the chance to discuss issues and voice fears that other members of the family are too close to. Parents may find that they confide closely in certain 91


The TOF Child friends and down-play things with others in order to preserve a 'normal' social life. "Many of my friends are often overconcerned about her health and probably feel I should 'wrap her up warmer' , others are very supportive and lend an ear when I am down." Friends can often seem overprotective: "Friends have been concerned and wanted to help, however, they can be overpowering. You may need time alone considering all the stress involved. However, it helps to talk." Entirely the wrong kind of response can also be given, although well-meaning. This is demonstrated by the following words from a TOF parent: "I gave my sister something to study when she was training to be a nurse." Even when the right response is given, at times it just isn't enough. "We have a few close friends who are really good and understanding. Most people don't have any idea what it's like however and mothers commenting on his size and speech etc., or trying to compare drives me insane! Parents with healthy children will never understand the feeling of seeing your child suffer with a long-term condition." The support gained from friends is crucial. However, in order to avoid embarrassment, any particular difficulties which the TOF child has should be explained; siblings can also be encouraged to explain about the TOF condition to their friends. "Though Simon is now fine, his regular mealtime vomiting was unpleasant. As a family we soon became used to this, yet I remember feeling embarrassed by my friends' reaction to him, as it was this that made me realise he was

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`different' . A few months ago Simon was discharged from hospital and the entire family felt a sense of relief. Though we've always accepted him the way he is, that occasion made us appreciate how lucky we are that he now leads a totally normal life." The opportunity to meet with a group of other TOF parents – especially those whose children are older so that they can pass on the benefit of their experience – can be very helpful to parents who feel on their own. "The contact I have had with other TOF parents with older children, breast feeding counsellors and 24 hour contact with the nurses at Great Ormond Street has been extremely useful. During visits home in-between hospital stays I have taken Amy to a couple of postnatal classes at which I have managed to be open about her condition, gaining interest from outsiders. This has been very important as it is too easy to feel different and alone with suppressed emotions!"

Growing up Moving on to playgroup constitutes another milestone, as does nursery and school. This book will be valuable to these institutions. "Playgroup were marvellous and managed well without complaint. Nursery was a disaster – they made a huge deal out of Nicholas problems and I got quite depressed about his condition and my ability to cope. School have been marvellous. The school doctor got a bit overexcited, probably because she sees so many ordinary children. Because we moved just before Nicholas started school the other parents do not know him and I get the feeling he is regarded as very


The TOF Child naughty by other mothers. (He isn't, he's just astonishingly lively)." "Eleanor started primary school when she was four-and-a-half and I had briefed her teacher about TOFs with the aid of the helpful TOFS Group leaflet. They all must have read it and t a k e n i t o n b o a r d b e c a u s e s o m e months later, I heard that the dinner ladies paid particular attention to the fact that Eleanor must drink plenty of fluid while eating!" As the child grows up, details can be added to the explanations. Up to at least 7 or 8 years of age, demonstrations will aid a child's understanding, and help them to cope with his/her difference. Some children will find this difference hard to cope with, especially as they grow older.

"Philippa feels left out sometimes, as she can't eat the same as her friends. At school she has a lunch box, as do the majority of six-year-olds, so no-one is any the wiser. She misses eating chips and looks forward to being older when she should be able to manage them. She doesn't want to stand out as being different from the other children." "Philippa's problems have not affected her schooling, apart from occasional time-off for check-ups. One friend's mum daren' t have Philippa for tea in case she gets stuck, although I think the fact that the children can be argumentative perhaps had more to do with it. She's OK at parties. I used to look to see what was on the menu and point out to Philippa what not to have. She now knows for herself what to avoid." Other children are capable of being open about their condition.

"Charlie had a foley catheter for a long time before getting a gastrostomy button. The day after his button was fitted he went to nursery determined to show all his friends. When I collected him later in the day it was to be told by the staff that Charlie and his button had kept the other children quiet for some time. Apparently, they had all disappeared into the play house and were found lying on the floor, T shirts pulled up, using cups and saucers as 'buttons' so they could be like Charlie. Shoe laces were removed and used as extension sets and kettles in place of 50ml syringes. I never expected anything like this level of acceptance from Charlie's peers and he was so pleased that everyone liked his new button – what more could we ask for from the under fives?"

Moving on The strain of a TOF child can be enormous on families. Different aspects of the experience stand out for different families.

"All the problems made me much closer to my TOF daughter than I might otherwise have been and the memory of that time remains to get me over 'teenager' crises." "Something out of the ordinary seems to polarise people – they either embrace the challenge with enthusiasm (or welldisguised fear) or don't want to try and understand or accommodate." "I have a very close, caring relationship with Lorenzo (TOF/VACTERL) as we have been through so much together. I think Joe, Lorenzo's brother, suffered slightly because of all the extra

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The TOF Child attention Lorenzo got. We still find it difficult to cope with, it has affected our lives very deeply; we are all closer and are all involved in Lorenzo's care. Friends try and understand but it's impossible – they don't understand our continual underlying anxiety." "I was frightened to be left alone with Ryan. He would choke and take his breath, so my daughter could not consider going back to work at all. All in all it has made us closer, but been very stressful on us all." "Even now, when he is so fit and well, we often look at him and remember what happened. It never leaves you." Nonetheless, families come through the challenges and learn to come to terms with and even to cherish the special nature of the experience. "I think that we are a closer family because of the trauma of having a TOF child." "Alistair was our first child and we tended to feel he was very special to us because of his condition and problems. The arrival of our second child showed us that the way we felt was because they were ours, not because there were problems. I did have to learn to relax and cope with Alistair's problems and this probably made me more relaxed with our other two children, but I think this is probably true with the oldest in every family – problems or not. We have moved house since Alistair had much of his surgery and went through the main recovery phase. If the subject arises, we explain what Alistair was born with and the main reactions are interest in the condition and s y m p a t h y a t w h a t h e h a d t o g o through. We have held regular coffee mornings for TOES in the village in an

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effort to raise people's awareness, but talk about it less as Alistair gets older – he finds it embarrassing to be singled out, as any teenager would."

Summary In summary, although a TOF child is different, and family life is very much affected by the arrival of a TOF child, it is important that the child is not lead to feel different – at least not so far as possible. The anxieties associated with the condition can have an enormous impact on a family but friends and relatives together with the backing of the medical team can help by offering practical help and emotional support. Patience and understanding may be required for others to accept the condition, but the rewards of such initial efforts are well worth it – not only for the parents' peace of mind and well-being, but also for the child's development in the long-term. If help is needed, ask for it. The term 'a TOF' – which is often used – can too easily become a 'label' for what is in many ways a normal child with a particular difficulty, born to live a fulfilling and enjoyable life: "I was very scared, especially as he choked quite badly the first time I looked after him. I panicked but once he's done it to you know what to look out for and to expect the next time. It never stopped me looking after him or treating him any differently."


The TOF Child

Long Term Outcome; Results of a Follow-up Study PAJ Chetcuti DM FRCP FRCPCH, Consultant Paediatrician, The General Infirmary at Leeds Considerable information has been provided on the possible problems encountered in children born with TOE This gives rise to two questions ... how common are these problems and do they improve as the child gets older? These questions were answered by a detailed follow up study from Melbourne, Australia in 1987. A total of 366 patients who had undergone TOF surgery (from the first successful operation in 1948 to those of the mid-1980's) were invited to attend the hospital for review. Over 300 attended and half were adults. Patients and/or their parents were asked about their current health and previous problems; height and weight measurements were taken, a clinical examination made and lung function tests performed.

Feeding One in 3 under 5 years of age experienced feeding difficulties with food sticking and vomiting but this improved dramatically in older children. Two thirds had been re-admitted to hospital due to swallowing problems, mostly in the first 5 years of life; just under half had needed a minor operation to stretch a narrowing in the oesophagus at the original operation site (a dilatation procedure). Two thirds of adults stated that they were aware of mild swallowing difficulties, however this did not interfere with what they ate and they were able to manage such

difficulties by drinking fluid with their meals. Only one adult had a narrowing in his oesophagus which needed a dilatation procedure.

Chest Problems Nearly half had been admitted to hospital with 'noisy' breathing and chest infections; again the majority of these episodes were in the first 5 years of life. The characteristic `TOF cough' was present in three quarters under 5 years of age, but became less frequent and less severe in older children. Just under half the adults said that whenever they coughed it was still the harsh brassy cough, but that this did not trouble them at all. Minor chest infections occurred in only 1 in 5 adults, but responded very quickly to antibiotics and did not require hospital admission. One third of adults occasionally experienced a `wheeze' (a whistling nose coming from the chest) but in over half this was because they had asthma and was unrelated to the operation.

Exercise, Education and Lifestyle Two thirds of older children and adults felt that they were extremely fit and regularly took part in sports where t he y c o mpet ed eq ual l y wi t h t h ei r peers. The rest reported only a slight reduction in ability to sustain vigorous exercise compared to their peers.

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The TOF Child 40% of 5-10 year olds missed more than one week of school per year because of illness compared to 20% of 10-15 year olds. Only 10% of adults missed more than one week of work because of any illness. They were involved in a wide variety of occupations and all said that their operation(s) had not interfered with their choice of career. The majority of adults were married and their 15 children were born with no significant health problems.

Height and Weight The height measurements in the group were similar to children and adults who had not experienced any major illnesses. 10% were extremely thin, but these were nearly all under 5 years of age, suggesting that nutrition and weight gain improved in older children and adults.

Lung Function Tests showed that 1 in 10 had slightly reduced lung volumes and lin 5 had slight obstruction to the flow of air out of the lungs. The patients with these findings were in no way disadvantaged and led normal lives.

Furtheimore, they grow up comparable to their peers and their achievements as adults are not impaired by their earlier problems.

Quotations from TOF s, their families and friends and medical professionals "Despite Jacob's continuous hospital admissions during his first year (and his inevitable small size), he makes up for this in strength of character and cheeky charm. He is already walking and is incredibly curious and determined." "It has undoubtedly been a very difficult time – Jacob was our first child and we spent much of his early life in hospital. He still doesn't eat and is slow to thrive. Despite all this he is our complete and utter pride and joy and we love him with a surging protectiveness that is hard to describe."

Summary

"Hannah is a lively 2 year old now, and although she has regular 'sticking' and 'choking' instances, and a cough that everyone remarks upon, we enjoy a wonderful 'normal' family life. We now have another daughter, Rebecca, who seems perfectly healthy and we look forward to a positive future."

A number of children born with TOF will develop feeding difficulties and chest problems. These problems are always at their worst in the first 2 years of life and after 5 years of age are uncommon. Some parents experience great difficulties in these early years and may need considerable support.

"James is a very lively, funny boy who enjoys every minute of his life and perhaps doesn't take things for granted, like so many of us do, without realising it often enough. We're so lucky to have James. James is a perfect example to show that this condition can be overcome with the best possible outcome."

However, this very large study – the largest of its kind on TOF follow up in the world – is very reassuring. It shows that the majority of children are able to participate fully in sport and to live up to their full academic potential.

"I believe she is some sort of 'miracle' child to come through all she had to suffer in her baby and infant life. She is very special."

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The TOF Child "Matthew is now nearly eight. A strongwilled energetic lad who is madly keen on football. TOF has not held him back and he is a picture of health. He is a real success story and an encouragement to all TOF parents." "Ben has been relatively healthy for a TOF. He has been dilated 3 times –Oxford, Bristol and Montreal, Canada. Fortunately we were well insured!!! He liked to visit a variety of hospitals! Winter coughs were a major problem until we discovered a wonderful GP who liaised with Oxford. We always kept a low dose antibiotic ready and used it when and if we needed it. He seemed to grow out of it at about 7 years as our Consultant had predicted although I never really believed it could happen. He is now 10 years old. Happy and healthy. He still gets stuck occasionally, but pushes it down with a drink. He represents the county in gymnastics, plays the trumpet and leads a normal life. The traumas were many along the way but he's been worth every minute. We also have an eight year old daughter who has no problems at all."

Above: Sandra Kay Lawton at age 3 years.

"For my second baby I was booked into a different hospital where they had the facilities for high-image scanning in order to look out for a possible TOF, and at about 29 weeks we saw our baby actually swallowing! In our heart of hearts we were not entirely convinced he was fine until he was born and breastfed very efficiently within 45 minutes of being born." "Matthew (7) is obviously aware of his condition. We have never made a big issue out of it and have not therefore explained the what TOF actually is. He still thinks the scars came from when he rescued his Dad from sharks in the sea! We will explain it to him, quite openly, when he has a more understanding mind."

Above: Christine Micolaud, who was born with TOF/OA, photographed on her wedding day. She is now mother to two chidren.

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The TOF Child

VACTERL R Lee BMedSci, Senior Medical Student and L Kapila OBE FRCS, Consultant Paediatric Surgeon, Queens Medical Centre, Nottingham.

WATER' is an acronym for: Vertebral (spinal) defects Anorectal atresia (failure of the anus and lower end of the gut to form) Tracheo-oesophageal fistula with or without Esophageal atresia (American spelling) Renal anomalies (abormalities of the (kidney) and radial limb dysplasia (abnormal forearm development). The relationship between these congenital anomalies was first noted in 1968, when it was called Say-Gerald Syndrome. Other synonyms have included Kaufman syndrome, PIV and PIAVA, but the term which is currently favoured (and is replacing VATER) is VACTERL. The extra letters stand for Cardiac (heart) and Limb defects respectively. They were added because it is now appreciated that over 70% of affected children have heart problems, and limb abnormalities are not always restricted to the forearm, with 40% of cases having defects of the foot or leg.

Diagnosis To qualify as a `VACTERL' child, three of the seven components mentioned above must be present. There may also be any number of other characteristics which occur more frequently in affected children than the rest of the population; these include ear abnormalities, genital anomalies, cleft lip and/or palate, thumb abnormalities, various changes in gut development and i n t he f et us, the presence of a single artery in the umbilical cord (normally there are two). No individual

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is likely to display all these features and no two individuals are likely to be affected in exactly the same way.

Cause It is estimated that for every 6,250 births, one child will have VACTERL. This is rare compared with many other conditions; one baby in 2,500 is born with cystic fibrosis, and one in 700 has Down syndrome. Large numbers of patients are required to scientifically study any medical condition, so the small numbers of children with VACTERL, and their wide variety of features, has made research difficult. Consequently, little is known. Growth of the fetus in the womb is enormously complex. The original two cells (the sperm and the egg) which combine and develop into a new individual divide, and their progeny divide and so on, until the millions of cells which make up a human being are formed. This is not just a process of multiplication, and different cells take on different jobs. Large groups of cells will become muscle, others will make up bone and eventually every type of cell in the body is represented. Ensuring that the right cells go to the right place at the right time is a delicate task; in spite of the massive scientific efforts aimed at understanding the intricate mechanisms involved, many pieces of the jigsaw are still missing. The cause of VACTERL is unclear. One theory suggests that cells are disrupted at an early stage of development. There are only three types of cell


The TOF Child in the three-week-old fetus, one of which is called a mesodermal cell. These cells go on to form the gut wall (including the oesophagus), the kidneys and bone (including the spine and the skeleton of the limbs). In principle, a change in a mesodermal cell could result in a change in any of these body parts, which would help explain the wide variety of abnormalities present in VACTERL. Efforts have been made to identify agents, such as drugs, which could adversely affect these cells, but so far nothing has been proved. It is important to stress that VAC _____ IERL is not an inherited condition. This means that the future offspring of a family with one VACTERL child have no greater chance of having VACTERL than the children of unaffected families. There is, however, an exception to the rule. Chromosomes are the inherited structures of DNA which determine everything about an individual. Two chromosomal abnormalities (the more common of which is Edwards syndrome) may result in the features of VACTERL –two rare cases in which the cause of VACTERL is known. In order to exclude this, the chromosomes of all VACTERL babies are examined.

Antenatal Diagnosis The regular use of ultrasound to examine the fetus is greatly increasing the number of abnormalities detected before birth. Many VACTERL babies will not be picked up during a routine scan, but in pregnancies which merit detailed ultrasound investigation it is more likely that skeletal abnormalities, kidney defects and a single umbilical artery will be seen. Perhaps, as ultrasound scanning continues to improve, ante-natal diagnosis of VACTERL will become the norm.

Symptoms and Treatment VACTERL children are categorised as TOFs because this is a cardinal feature of the condition, but their problems and hospital management differ because of their multiple abnormalities. Only 60% of all `VACTERLs' will have a TOF to repair.

Vertebral Abnormalities Vertebrae are flat bones which are stacked on top of each other to form the spine. If they are deformed, an abnormal spinal shape may result (` scoliosis' or `kyphosis'). Abnormal bone development may also be accompanied by abnormalities in the associated muscles and nerves, and these children can appear slightly lopsided if the left and right sides of the body are affected unequally. Fortunately, these deformities are often mild. Treatment of an abnormally shaped spine depends on the severity of the deformity. If minor, no intervention may be required. More severe cases often necessitate use of a brace (a contraption worn by the child which exerts a force in order to straighten or prevent further curvature of the spine). This is a long term option and can be worn for several years. Gross distortions require surgical correction as they may cause severe disability, perhaps decreasing the size of the chest and therefore the space available for the lungs to expand. This is a major undertaking with a long post-operative course.

Scoliosis

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The TOF Child Anorectal Atresia This is a broad term which describes a range of abnoinialities. At one extreme, children have an intact bowel with a blind end Clow imperforate anus'). At the other extreme, the bowel stops quite some distance short of what should be the anal opening, and there are often abnormal connections between the bowel and the bladder or vagina. A low imperforate anus is treated by a single surgical procedure soon after birth which has few long-term complications. The more severe (high) case requires two or three separate operations, the first of which creates a `colostomy.' This is an artificial opening in the skin which is attached to the bowel on the inside and a plastic bag on the outside. It enables the child to defaecate and is easily managed by regular bag changes. In subsequent operations the bowel and anus are reconnected and, after healing, the colostomy is closed. Continence will develop if the surrounding muscles and nerves are able to function adequately.

Above: Ano recta l Atresia an d Hypo spad ia s. This child does not have an anus (anorectal atresia) and his urinary orifice is situated on the under-surface of the penile shaft (hypospadias). He also has an abnormal connection between the bowel and bladder; faeces are therefore seen at the urinary orifice.

Cardiac Abnormalities `Ventricular septal defect' (VSD) is the commonest type of cardiac abnormality (abnormality of the heart), a c c o u n t i n g f o r m o r e t h a n t h r e e - quarters of all cases. It is a hole in the wall that separates the two large chambers of the heart. Consequently, the heart does not function efficiently. The child is symptom-free if the hole is small, but a doctor would be able to detect it. Moderate cases may cause breathlessness on feeding and recurrent chest infections, but these problems tend to subside with age. Babies will be short of breath at an earlier age with severe defects and often sweat. They may be underweight and become severely ill.

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With all VSD's there is a greater risk of heart infection, and in mild cases the only treatment is a course of antibiotics given before surgical or dental procedures. In moderate cases, children can accumulate excess body fluid, and this is usually controlled wi t h t a bl et s . S ur ger y t o cl ose t he defect is largely reserved for the severe case which does not respond to medication. This is best done as a single operation, during which the function of the baby's heart and lungs are taken over by a machine. There are many other heart defects which may occur in isolation or accompany a VSD.


The TOF Child

b o d y

Tracheo-Oesophageal Fistula with Oesophageal Atresia Symptoms and treatment do not differ from other TOFs.

Renal Anomalies These fall into two main categories; total failure of one or both kidneys to form, and 'other' anomalies. Absence of both kidneys has sinister implications and may be detected antenatally by ultrasound. A single absent kidney is however entirely symptomless, because the remaining kidney can fully compensate for the deficiency. The 'other' category is vast, including abnormally small (` hypoplastic') kidneys, abnormally shaped kidneys (e.g. 'horseshoe kidney'), abnormally placed kidneys (e.g. 'pelvic kidney'), abnormally arranged kidney cells (e.g. `polycystic kidney') and abnormal structures which communicate with the kidney (e.g. an abnormal blood s u p p l y , o r a n a b n o r m a l s h a p e o r number of the tubes (ureters) which run between the kidney and bladder). These are not always associated with malfunction, but problems arise

when the kidney cells do not work efficiently, either because they never developed properly or because they have been damaged. The latter is commonly caused by an obstruction to urine flow, particularly at the junction of the kidney and ureter. The resultant backlog of urine is often associated with infection and the baby may have diarrhoea, a fever, vomiting and be slow to gain weight. Antibiotics are prescribed to prevent or treat infection a n d t h e o b s t r u c t i o n i s r e l i e v e d surgically. If only one kidney is involved the other will always compensate.

Normal kidneys Line of rib cage Right kidney'

Left kidney

Spine

Ureters

Pelvis —

Bladder

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The TOF Child

Renal abnormalities Unilateral agenesis (one kidney)

Pelvic kidney

Hypo plastic kidney

Horseshoe kidney

Crossed renal ectopia

Ureteric duplication

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Polycystic kidney

Below:

Radiograph of a grossly enlarged kidney filled with contrast. Urine is produced by this kidney, but cannot leave via the ureter because of an obstruction.


The TOF Child Limb Abnormalities

The VACTERL Child

The forearm (the part of the arm between the elbow and wrist) contains two bones, the ulna and radius. Partial or total failure of the radius (and the muscles which attach to it) to develop is frequently associated with VACTERL. Characteristically this causes the hand to lie at a right-angle to the forearm and usually the thumb is also malformed or absent. Considerable deformity and disability may result.

Hospitals are a second home for VACTERL children. They receive intensive medical attention, both to treat the immediate problems and to monitor their progress. This involves frequent consultations with a variety of specialist doctors, in addition to intensive general health surveillance, such as regular growth checks. The impact of such prolonged hospitalisation should not be underestimated. Although VACTERL children have comparable intelligence to their peers, their social integration and development is almost inevitably affected.

Initially, the affected arm is passively corrected with a splint. This is followed by an operation between the age of 6 and 12 months to realign the hand and forearm. If the thumb is absent a second procedure, called 'pollicization', is used to create a surrogate thumb by rotating the index finger. Physiotherapy is an important part of the rehabilitation process and a considerable degree of hand function can be restored. Further details about these problems are given in the following chapter.

Ultimately, the future of an affected child will be determined by the success of their treatment. Residual problems may cause long-term disability, the severity of which will vary between individuals and depends on the number and type of defects. In the majority of cases, continued medical support will be required, but this does not prevent a V A C T E R L c h i l d f r o m l e a di n g a n active, independent and fulfilled life.

Below: Radial club hand, a characteristic limb anomaly in VACTERL children.

References 1. Bayne L.G. (1993). Radial Club Hand (Radial Deficiencies) in `Operative Hand Surgery,' Churchill Livingstone, New York. 2. Beals R.K. and Rolfe B. (1989). `Current Concepts Review VATER Association. A Unifying Concept of Multiple Anomalies.' The Journal of Bone and Joint Surgery 71-A: 948950. 3. Buck-Gramcko D. (1985). aadialisation as a New Treatment for Radial Club Hand.' The Journal of Hand Surgery 10A: 964-968.

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The TOF Child 4. Chen J.M., Schloss M.D. and Laberge J. (1991). 'Extensive Upper Aerodigestive Tract Anomalies in `VACTERL' Association.' Archives of Otolaryngology, Head and Neck Surgery 117: 1407-1410. 5. Jablonski S. (1991). Jablonski's Dictionary of Syndromes and Eponymous Diseases, Krieger Publishing Co.: 624. 6. Khoury M.J., Cordero J.F., Greenberg F., James L.M. and Erickson J.D. (1983). `A Population Study of the VACTERL Association: Evidence for Its Etiologic Heterogeneity.' Paediatrics 71: 815-820. 7. Merlob P. and Naor N. (1994). `Drug Induced VATER Association: Is Dibenzepin a Possible Cause?' [Letter]. J Med Genet 31: 423. 8. Morris P.J. and Matt R.A. (1994). `Oxford Textbook of Surgery,' Oxford University Press.

9. Sadler T.W. (1990). `Langman's Medical Embryology,' Williams and Wilkins, Baltimore. 10. Say B. and Gerald P. (1968). `A New Polydactyly/ImperforateAnus/Vertebral-Anomalies Syndrome?' [Letter]. The Lancet 2: 688. 11. Strickland J.W. and Kleinman W.B. (1993). 'Thumb Reconstruction for Congenital Absence' in 'Operative Hand Surgery,' Churchill Livingstone, New York: 2044-2073. 12. Weaver D.D., Mapstone C.L. and Yu P. (1986). 'The VATER Association. Analysis of 46 Patients.' Am J Dis Child 140: 225229. 13. Wiedemann H.R., Kunze J., Dibbern H. (1992). NATER Association,' in 'An Atlas of Clinical Syndromes. A Visual Aid to Diagnosis,' Wolfe Publishing Ltd.: 548.

Ben Gordon, VACTERL child Ben was born with TOF/OA, radial club left hand and a vertebra missing. Left; Ben aged 14 months. Below; his 4th birthday, after surgery on his arm; Ben is in the middle near the base of the photograph.

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The TOF Child Useful Resources and Support Groups for VACTERL Information about VACTERL can be gained from TOFS office: additional contact details may also be available from your hospital.

Association for Children with Heart Disorders

REACH: The Association for Children with Hand or Arm Deficiency

Postal contact through umbrella group, Children's Heart Foundation (see below) Tel: 01706 213632 (Helpline) Email: information@heartchild.info Web site: www.heartchild.info

PO Box 54, Helston, Cornwall TR13 8WD Tel: 0845 1306 225 Email: reach@reach.org.uk Web site: www.reach.org.uk

British Kidney Patient Foundation

Sacral Agenesis Contact Group

Bordon, Hants GU35 9JZ Tel: 01420 472021/2 Web site: www.britishkidney-pa.co.uk

15 Elizabeth Gardens, Dibden Purlieu, Southampton, Hants SO5 4NF Tel: 023 8084 2661

Children's Heart Foundation

STEPS

52, Kennington Oval, London SE1 1 5SW Tel: 0808 808 5000 (Freephone 9.30am – 9.30pm)

Association for People with Lower Limb Abnormalities Lymm Court, 11 Eagle Brow, Lymm, Cheshire WA 13 OLP Tel: 0871 717 0044 (Helpline open Monday-Friday, 9.30am - 4.00pm) Email:info@steps-charity.org.uk Web site:www.steps-charity.org.uk

Email: chf@dircon.co.uk Web site: www.childrens-heart-fed.org.uk

Contact a Family (source of contact details for other medical support groups and charities) 209-211 City Road, London EC1V 1JN Tel: 0808 808 3555 (Helpline open loam-4pm, Mon-Fri). On-line helpline through web site Web site: www.cafamily.org.uk

NASPCS (National Advisory Service to Parents of Children with a Stoma)

Tracheo-Oesophageal Fistula Support (TOFS) St. George's Centre, 91 Victoria Road, Netherfield, Nottingham NG4 2NN Tel: 0115 961 3092 Email: info@tofs.org.uk Web site: www.tofs.org.uk

51 Anderson Drive, Darvel, Ayrshire KA17 ODE Tel: 01560 322024 Email: john@stoma-freeserve.co.uk Web site: www.naspcs.co.uk

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The TOF Child

Radial Deformities in VACTERL By Simon P.J. Kay, FRCS, FRCS (Plastic Surgery), Consultant Plastic Surgeon, St James University Hospital, Leeds. The VACTERL anomaly includes the component 'radial.' This refers to radial dysplasias which affect the forearm and hand. In order to understand these conditions, one must be familiar with the basic anatomy of the forearm.

Nomenclature The forearm is made up of two bones; the ulna which hinges with the upper arm humerus, and the radius which swivels around the ulna as the hand rotates. Because the hand can rotate from the elbow so that the palm faces forward or backwards, the borders of the forearm are not named inner and outer but are named after the underlying bone; radial or ulnar. The thumb therefore lies on the radial border of the hand while the little finger lies on the ulnar border.

Forearm Viewed from the Front

`Dysplasia' simply means abnormal development. In radial dysplasia there is a variable degree of abnormal development on the radial border of the forearm and hand. Radial dysplasia may occur in one or both arms, and is particularly commonly associated with the VACTERL anomaly, but may occur on its own or in association with other syndromes. The resulting deficiency is best considered in terms of the forearm deformity and the hand defonnity. These do not necessarily correspond; a major abnormality of the thumb may be present with a minor abnormality of the forearm (or vice versa).

The Forearm Anomaly: Radial Club Hand. The normal hand articulates with both t he r adi us and the ul na at the wrist. The articulation with the ulna is relatively small and the radius provides more of the support for the hand. In radial dysplasia the soft tissues and flesh of the forearm are affected as well as the bone. The arrangement of muscles and nerves may be abnormal and some are commonly absent. The degree to which the radius is abnormal is variable; in the most Left: Arrangement of forearm bones. Note how the radius rotates around the ulna and each border of the hand is named after the underlying bone when the palm is facing forwards and the elbow is by the side (the so-called anatomical position).

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The TOF Child severe cases the radius is completely absent and the elbow joint may be disturbed or even fused with no movement possible. In the mildest of cases the radius is merely slightly smaller than the other side and there is minimal deviation at the wrist. Between these two extremes there may be a small remnant of radius, or the radius may be only partially formed. In any case where the distal part (hand end) of the radius is absent, there may be an abnormal bar of gristle connecting the remnants of the radius to the hand. This gristle remnant is known by the German word `anlage' and has a very limited ability to grow. It is attached to the radial border of the hand and wrist; as the ulna grows in the mother's womb, lack of growth in the radial anlage draws the hand into a deviated "club" position.

Anlage Results in Deviation of the Hand with Growth...

anlage tethers the wrist, meaning that the ulna pushes the wrist further and further into deviation as it grows in length. Another important component of the deviating force is muscle action. Once the wrist has begun to deviate, muscle activity will tend to exacerbate the deviation as the wrist is levered about its fulcrum or point of contact with the ulna.

Muscle Pull Causing Hand Deviation

Fulcrum

Remnant of radius

Muscle pull

Treatment of the Forearm Treatment of the wrist and forearm may not be seen as a priority at the time of birth. However, by the time the child reaches adolescence, many of the other anomalies in the VACTERL syndrome will either have been successfully addressed or be of little consequence. The wrist and hand may then be the most significant and enduring stigma of the condition and may be the only part that is causing any form of disability. The degree to which the hand and wrist deviate at birth and thereafter depends on the degree to which support is lacking (i.e. the nature of the remaining bony platfoiin on which the hand sits) and the degree to which the

Surgical treatment is by no means automatically indicated and there are complex decisions to be made along the way before deciding what treatment may be appropriate.

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The TOF Child The Immediate Neonatal Period

Surgical Treatment of the Elbow

It has been shown that splinting and passive stretching exercises fo r the wrist and elbow can not only maintain whatever motion is already available, but can also improve an initially poor range of motion. Such exercises may also lessen the degree of deviation at the wrist.

If the elbow is completely rigid, treatment may not be possible. In some cases it is possible to surgically release the structures at the back of the elbow or any bony bridge between the forearm and the upper arm (synostosis) in such a way as to allow some assisted or passive motion to be restored. Thereafter voluntary movement and physical strength may be restored to the elbow by transferring a muscle from the wall of the chest. This can be very successful in restoring to the child the ability to bend and sometimes to straighten the elbow. However, it is not indicated in all cases, depending on the initial condition of the elbow.

The application of splints to a neonate's arm is extremely difficult and can be very trying for both the physiotherapist and the mother. For this reason many surgeons prefer to guide their patients in gentle but firm and frequently repeated passive stretching exercises in the wrist and elbow. If these are begun early in a child's life they will be taken as normal and will not provoke distress. Most children with radial dysplasia have good or normal flexion and extension (bending and straightening movements) in the elbow. In patients where the range of motion is restricted, any improvement as a result of therapy is welcome, even if the child is unable to move the elbow voluntarily. Once the flexibility of the joint has been regained, the ability to move the joint can be restored by surgery, provided the joint remains supple and mobile. Physiotherapy alone will not resolve the wrist contracture and deviation but this should not discourage parents from an exercise r e gi men . A n y i mpr o ve me nt i n t he range of movement in the joint will make subsequent surgery and splinting more effective and less complex.

Many surgeons prefer to delay this surgery until the age of three or four years when the structures in the region of the elbow are more mature, while others prefer to restore elbow bending at as early an age as possible in order to facilitate subsequent decisions about the management of the wrist.

Surgical Treatment of the Wrist Much of the surgical management of the wrist was pioneered during the Thalidomide epidemic of the 1960s. Not all children should have the wrist straightened; in particular those children with VACTERL anomaly having rigid, straight elbows should only have wrist surgery under unusual circumstances and after careful discussion. If the elbow is unable to bend, the presence of a bent wrist and short forearm can be an advantage in personal hygiene and feeding. This kind of surgery is also relatively contraindicated in cases with other serious complications.

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The TOF Child Surgical endeavours to straighten the wrist have undergone many advances and changes. In principle, the tight structures which are tending to deviate the wrist (the skin, the radial anlage, the abnormal muscles, and the tight joint capsule and lining of the joint) are divided or lengthened and the wrist is therefore able to rotate straight on the end of the ulna. In practice, the wrist can be very tight and difficult to free up. Several solutions to this problem have been found, including removing bone from the wrist so that the hand can sit straight on the end of the ulna, or removing a slot of bone from the wrist so that the wrist fits like a cap over the end of the ulna.

The sensitive 'growth zone' at the tip of the ulna can also be damaged by excessive pressure; for this reason it is better to remove bone from the wrist than to force the wrist bone over the ulna under great compression. In recent years there has been a move towards distraction lengthening in which a frame is placed on the hand and forearm in such a way that pins through the ulna and pins through the hand can be moved apart gradually (1mm a day maximum) so as to gently stretch and 'grow' the hand into a straighter position prior to surgery. The result of this is that there is little or no compression when the hand is finally surgically straightened on the wrist.

The latter procedure is known as centralisation and is preferable because removing bone from the ulna could affect growth. The end of the ulna is where its longitudinal growth occurs; removal of or damage to the end of the ulna may therefore result in an even shorter forearm than would normally be the case.

This procedure has proved very effective although in many units use of the technique is only in its infancy and surgeons are still learning the risks and benefits of the procedure. Below. Radiograph of a child with a distraction device on both sides of the wrist. Sometimes these are only put on one side.

Centralisation

The surgeon may be unable to straighten the wrist...

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The TOF Child

If little or no bone has been removed in order to accommodate the hand and wrist on the end of the ulna, then the hand may be pushed slightly further across towards the ulnar border of the forearm and the tight muscles of the wrist reorganised so as to balance the hand on the end of the ulna. This procedure is known as radialisation and has the theoretical benefits over centralisation of resecting less bone, being less likely to interfere with growth, and allowing more mobility at the wrist (because the muscles are restored and the ulna is not placed in the slot within the wrist). Protracted splinting may be necessary after centralisation or radialisation. At the time of surgery a pin is placed through the wrist and the ulna and this serves as an 'internal splint' which may remain in place for a year or even more after surgery and may be augmented by an external splint. It may be necessary to continue splinting for many months or even years before the ulna gradually broadens and becomes a more stable platform on which the wrist may balance.

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In practice it has proved very difficult to obtain reliable and reproducible results with radialisation. In both centralisation and radialisation further surgery for rebalancing may be necessary even quite early after the first procedure and certainly is not unusual at least once during the growth of the child. At the time of radialisation or centralisation there is a deficit of skin on the radial border of the wrist and an excess of skin on the ulnar border of the wrist. Various operations have been devised to redress the balance between these two areas but all the procedures result in scarring on the back of the wrist and in some cases may give rise to slow healing in at least one part of the wound. This is not usually painful or disturbing for the child. At the time of radialisation or centralisation the surgeon will be able to inspect the tendons which pass from the forearm to the hand as well as the muscles and soft tissue structures in the hand itself. This may be important in planning surgery to the hand later.


The TOF Child Surgical Treatment of the Forearm Bones Frequently the restrictions causing hand deviation also affect the growth of the ulna bone. When the radius is partially or completely absent and there is an unyielding anlage connecting the elbow to the wrist, then not only will the wrist deviate but the ulna may be forced to grow in a curve (i.e. to 'bow'). This bowing can become extreme and result in a 90° bend in the forearm. It may also result in a twist in the forearm because of the complex arrangement of the anlage. These abnormalities may lead to an apparently shorter forearm than is truly the case. At the time of the operation for radialisation and centralisation of the wrist, the surgeon may choose to straighten the forearm bones by rearranging wedges of bone within the middle of the arc and the ulnar. These will be held in place by the same pin that holds the wrist.

There has been some discussion about the pros and cons of distraction lengthening of the foreatm after the wrist has been centralised (this is in distinction to the distraction lengthening mentioned previously, which serves to straighten the wrist prior to surgery and which may be undertaken from a very early age –even in the neonatal period). Distraction lengthening of the forearm bones is generally considered to be an option for later in the child's life and the benefits of this may, in some patients eyes, be outweighed by the disadvantages. These need to be discussed carefully with the surgeon undertaking the procedure, and include minor infections, pain, discomfort and scarring as well as the possibility of poor healing and function. It should be recognised that any distraction lengthening may need to be repeated a number of times while the child is still growing.

The Hand Anomaly In radial dysplasia, the major anomaly in the hand is a variable degree of hypoplasia of the thumb and abnormalities in the long tendons which pass to the remaining fingers. Hypoplasia simply means undergrowth and hypoplasia of the thumb has been classified by the German surgeon Blauth as follows: Type I: slight reduction in size but all structures normal. Type II: small thumb with abnormalities of the muscles and tendons, instability (wobbliness) of the middle joinfof the thumb and a tight restricted web space between the thumb and index finger.

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The TOF Child Type III: small skeleton of the thumb, abnormalities of many if not all of the muscles, abnormal stiffness in all of the thumb joints and a highly abnormal first web space. The position of the thumb may also be abnormal. Type IV: a 'floating' thumb attached by only a thread. Type V: total absence of the thumb. The abnotnialities of the thumb are surprisingly often overlooked when they are mild at birth. The decision whether or not to treat the abnormality when it is found is not always straightforward.

Above: Type III thumb.

Treatment of the Hand Type I Type I hypoplasia needs no treatment since function is virtually normal.

Type II Type II hypoplasia often requires three elements in treatment, all of which may be undertaken at one stage. Firstly, the tight web space between the thumb and index finger is released with skin grafts or the transposition of skin from an adjacent area. Secondly, the wobbliness or instability of the middle joint (the metacarpophalangeal or MCP joint) of the thumb is addressed. This is either through ligament reconstruction or, in more severe cases, by making this joint rigid. The latter may sound drastic, however the main functional ability of the thumb comes from the joint at the base of the thumb and rigidity at even one or even both of the other joints is functionally preferable to floppiness or instability. Finally, the abnormality in the muscles of the thumb is addressed. Many surgeons believe that these abnormalities should be corrected at the same time in order to prevent

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Above: Type IV thumb.

Above: Type V thumb.


The TOF Child recurrence of the tightness in the first web space, which might otherwise occur if the thumb is not sufficiently active to 'exercise' the skin on the hand. Some surgeons prefer to rearrange t he l ong tendons of t he f or ear m t o allow the thumb to be moved away from the palm or to rotate across the palm while other surgeons prefer to move one of the small muscles from the little finger border of the hand in order to provide this function. On rare occasions, it is necessary to restore the long tendon that allows the thumb to bend; this is usually best left until such time as the child is able to cooperate with rehabilitation.

however it is an operation which is very well understood and has proven to be very reliable and reproducible. In cases where the index finger is normal and functional, a very good thumb results; the choice is therefore between a four fingered hand without a thumb or a three fingered hand with a thumb. So crucial is the function of the thumb to the function of the hand as a whole that most sur geons believe that the child is better served by the creation of a three fingered hand with a thumb. Cosmetically most surgeons also find such a hand less bizarre and noticeable.

Types III, IV and V In Type III the widespread nature of the abnormalities is such that one has to consider complete reconstruction of the thumb. Type IV and V patients have no option other than to do nothing or undergo such surgery. M a n y s u r ge o n s h a ve so u gh t t o avoid this and have subdivided Type III in order to identify those cases in which the existing inadequate thumb can be preserved in large part and elements of the skeleton and the joints replaced by microsurgical transplantation from the foot. Some of these pioneering works have proved successful but it is still to early to make widespread recommendations about these techniques. Instead, in appropriate cases, reconstruction of a very functional and aesthetically pleasing thumb can be completed by transferring the index finger. This operation is known as pollicisation. It may seem extraordinarily severe because it sacrifices an apparently normal index finger in order to construct a thumb,

Above:

The Type IV case shown on the previous page, following pollicisation.

Timing of Surgery Much has been written about the pros and cons of timing of the various stages of surgery. What is not in doubt in syndromes such as VACTERL and HoltOram anomaly is that life-threatening conditions take precedence. Under the best possible conditions many surgeons prefer to complete radialisation or centralisation of the wrist and straightening of the forearm (assuming a normal elbow) by six to eight months of age, leaving the child free to have a pollicisation procedure if appropriate (see below) at about one year to eighteen months of age.

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The TOF Child Where the wrist does not need centralisation or radialisation, the pollicisation procedure may be brought forward. Some surgeons have indicated that there is a penalty for delaying this surgical timetable. Others increasingly feel that such an opinion is based on assumptions rather than fact and that only the progressive deformity of the wrist needs to be treated early, after which the nonprogressive abnormality of the thumb may be treated at a more c o n s i d e r e d t i m e p r o v i d e d i t i s completed well before school years. Contraindications to pollicisation Not all children with a Type III or worse thumb should undergo a pollicisation procedure. Parental attitudes should be considered carefully and their wishes taken into account. Children with a normal hand on one side have a less imperative need for reconstruction on the opposite side. This is not to say that reconstruction should not be offered to those children; many experienced surgeons feel that cases in w h i c h o n l y o n e h a n d i s a f f e c t e d benefit just as much from surgery as those in which two hands are affected. More importantly however, the condition of the index finger and the other fingers is pivotal in deciding whether to reconstruct the thumb. The final functional quality of the pollicised thumb is determined mostly by t he i ni t i al qual i t y of t he i ndex finger. In some cases the fingers are incapable of independent movement, have abnormalities including duplications or deviations within the structure of the finger, or have stiff joints and poor movement. In these cases a poor thumb will result and pollicisation may not be indicated.

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Some severe anomalies of the forearm muscles and tendons noted at wrist surgery may also make the surgeon decide against pollicisation. An important relative contraindication is the 'occasional' or inexperienced surgeon. Pollicisation is an operation in which experience is of enormous benefit since the procedure is quite complex. Parents may feel reassured by seeing examples of other cases treated at the surgical centre. Pollicisation is contraindicated in the uncorrected wrist. This is because the radial border (thumb border) of the hand in the uncorrected wrist is not the side which first approaches an object. Such children almost universally have a prehension (grasping) pattern which uses the little finger (which is in their case the leading edge of the hand) as the thumb, meaning that objects are usually held between the little finger and ring finger. If the wrist is corrected relatively late in the child's development (perhaps because of late correction of the elbow) then the child may have become so accustomed to this pattern of grasp that after pollicisation the pattern is hard or slow to break and the child neglects the new thumb. The decision whether or not to offer pollicisation requires experience and judgement on the part of the surgeon. Dogmatic opinions about absolute contraindications to surgery in these cases are rarely valid and provided a good thumb is reconstructed following correction of the wrist and elbow most children of an appropriate age will revert to using the thumb rather than the little finger. Some children make use of both patterns of prehension; this is perfectly reasonable since it adds rather than detracts from the functional repertoire of the hand and also improving its cosmetic appearance.


The TOF Child

Late Reconstruction and Secondary Surgery Late reconstruction and secondary surgery are very common in radial dysplasia. Each element of the dysplastic forearm may need further help as the child grows. The elbow may need further help with bending, the forearm may need straightening or distraction lengthening later in the growth period, the wrist may need revision of centralisation or radialisation and, in about 25% of cases following pollicisation, the thumb may require muscle transfer to help with the opposition movement (rolling across the palm) of the thumb. The latter movement will never be as fluid and functional as in the normal thumb but can be very satisfactorily restored at pollicisation; even when restoration at the initial operation is not complete, subsequent muscle transfers may greatly improve this movement.

Summary Radial dysplasia as part of the VACTERL anomaly may seem the least of the child's worries at birth when tracheo-oesophageal fistula, cardiac anomalies or renal anomalies may be urgently requiring attention. After the other anomalies have been corrected, the radial dysplasia may however be more of a restriction for a child. Treatment of this condition is specialised and is best conducted in a surgical unit where the whole repertoire of forearm and hand reconstruction is available. Staged and repeated operations are often necessary, in combination with specialised physiotherapy (much of which can be done at home) and splinting. The functional and cosmetic outcomes are frequently rewarding, however depend not only the skill, experience and devotion of the whole team caring for the child (including the parents) but also the extent and severity of the original deformity.

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The TOF Child

The Role of the Professional; Who to Ask for Help by Suzanne Goodley SRN SCM, Family Care Sister, Nottingham Neonatal Service.

During pregnancy and maybe before, you will already be aware of a number of health professionals. The health professionals most of us know about are members of the primary health care team, which comprises the following (listed in alphabetical order):Community midwife General practitioner Health visitor Practice nurse When a baby is sick, premature or requiring surgery and therefore needing admission to a Neonatal Intensive Care Unit (NICU), additional professionals will be involved in the care of the baby. The following will be added to the above list (again in alphabetical order, so as not to indicate any one as being more important that another):Chaplains of various denominations Dietician Geneticist Hospital midwife Liaison health visitor Neonatal nurse / advanced neonatal nurse practitioner Neonatologist or paediatrician Ophthalmologist Paediatric surgeon Physiotherapist Radiologist Radiographer Social worker Speech therapist The roles of these medical professionals are now explained in more detail.

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Community Midwife The community midwife is trained to care for mothers and babies pre-natally, ante-natally and post-natally up to 28 days after delivery. During the course of the pregnancy parents will have come to know and trust the midwife; even where the baby needs to be moved onto a neonatal unit the midwife should still be aware of progress and will often remain in close contact.

General Practitioner (GP) This is your family doctor who you should already know. A GP cares for family health needs from his/her local practice.

Health Visitor The health visitor is a qualified nurse who has had extra training to practise as a health visitor and is responsible for you and your children until they reach school age. Their responsibilities include helping with health promotion/preventative medicine, running well baby clinics, dealing with social, emotional and environmental issues, and performing regular child assessments to monitor a child's progress with reference to recognised developmental milestones. Usually the health visitor will introduce themselves to a family antenatally – if not already known to you through previous children – but will otherwise be in contact either soon after delivery or once the baby is home.


The TOF Child Practice Nurse A general practitioner usually has at least one nurse attached to their practice who is responsible for nursing duties within the practice and may therefore be known to many of the patients. The practice nurse's involvements include well woman and man clinics, wound dressings, removal of sutures, immunisations and innoculations.

Chaplains of Various Denominations Most hospitals have a chaplain attached to them, if not a full chaplaincy service. They are there to give spiritual, emotional and practical support and are always on hand, day or night. If you wish to have your baby baptised they will be able to perform a simple service for you.

Dietician A dietician is trained in nutrition. Advice is often sought from them in caring for sick babies regarding the correct type and amount of feed to give to enable them to grow. Some babies have unique requirements after surgery and therefore need special diets.

Geneticist This is a doctor who specialises in genetics, the study of the genes and chromosomes in the many cells which make up each individual. Sometimes having a baby with an abnormality such as TOF can have genetic implications for you and your children, and therefore you will be referred to one of these specialists if either you make a request for this, or if the doctor believes it to be advisable.

Hospital Midwife The hospital midwife is trained in the same way as your community midwife, but works in a hospital

setting. She will be responsible for the mother's post-natal care while she is resident in the hospital. Postnatally a mother needs to be seen daily by a midwife for the first ten days, and then at less frequent intervals for up to twentyeight days afterwards, depending on circumstances.

Liaison Health Visitor This is a health visitor based within a hospital whose role is to communicate with other health professionals. They will normally inform the health visitors in the parents' local area that a baby on their case load is a patient on a neonatal unit, and then keep them informed and up to date on the baby's and family's well being. In some units they also act as a support person for the family, instead of the role of a family care sister.

Neonatal Nurse The nurses on a neonatal intensive care unit are trained nurses who have taken on additional training to be able to specialise in caring for premature and sick babies, and are therefore well qualified to care for your baby. Most neonatal intensive care units use what is called a 'named nurse' or `primary nurse' system. This means that, within a short time of your baby being admitted to the unit, a nurse will be named as the person with some increased responsibility for their care. Obviously this nurse cannot be on duty al l t he t i me, but wi l l i denti f y t he baby's and the family's needs, write an appropriate nursing care plan and ensure that this is carried out, sharing care with other nurses in her absence. Units work shift systems which vary from unit to unit, but you no doubt will soon become familiar with the system employed.

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The TOF Child A pa r t f r o m t he nur ses act ual l y caring for your baby on a day to day basis, some units have other specialist nurses to help care for you and your baby during you stay. These may be introduced as a 'family care sister,' a title which is self explanatory; the sister cares for the whole family, encompassing the entire scenario of having a sick baby, and all the emotions that entails. Other titles used for such a nurse might be 'paediatric community sister' or `liaison neonatal nurse,' but the aim of them all is the same. They will sometimes start their relationship with parents antenatally, introducing families to a neonatal intensive care unit prior to delivery, and will follow the whole family through to discharge home, ensuring that the transition from a

stressful neonatal intensive care unit to the hopefully relaxed atmosphere of home goes as smoothly as possible. In some neonatal intensive care units all babies automatically have their hearing tested (and it is hoped that in the near future universal testing of all babies born will be carried out.) The person performing this test is usually another neonatal nurse, who has undertaken further training to be able to test babies' hearing. This test is not harmful to the babies. Special equipment measures the response from the auditory (hearing) nerve to quiet sounds played into their ears. The test is done when the baby is asleep.

Advanced Neonatal Nurse Practitioner (ANNP or NP) This is a neonatal nurse who has several years experience and has undertaken further extensive training. This enables them to carry extra responsibilities, although how these are expressed varies between units. Not all units have ANNPs. Most commonly they are on the unit to give expert continuing clinical care of babies, often in a role very similar to that of the junior doctors. They may attend deliveries of sick babies to provide resuscitation, perform routine baby examinations on the postnatal word or work on the neonatal unit where they are able to provide advanced care for sick babies often in place of or alongside junior doctors.

Neonatologist or Paediatrician

Above: Lesley Brown, 1 year, who had a long gap OA and spent a long time in hospital; the nurses became very fond of her ...

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A neonatologist is a doctor who specialises in the new-born infant. A paediatrician is a doctor who specialises in all children, from newborn to adulthood. There will be a team of doctors on a Neonatal Unit led by a consultant –


The TOF Child either a consultant neonatologist of consultant paediatrician – and they are in overall charge of the baby's care. They usually make daily ward rounds and are often on the unit during normal working hours and when on call. Next in line are the senior registrars/registrars; these are senior paediatricians who are working towards becoming a consultant. The senior house officers (SHOs) are the junior doctors who, along with the nurses, are responsible for the day to day work and running of the unit. A l t h o u gh j un i or , t h e y a r e us u al l y trained in the speciality of neonatology or paediatrics. Medical students do not work in neonatal intensive care units, but may be present from time to time, just as observers.

Ophthalmologist This is a doctor who specialises in eyes. Babies that have required neonatal intensive care and needed oxygen therapy as part of their treatment will have their eyes checked routinely by an ophthalmologist.

Paediatric Surgeon

Radiologist This is a consultant doctor who specialises in the interpretation of radiographs (X-ray pictures) and scans e.g. ultrasonographs, CAT (Computer Assisted Tomography) scans, NMR (Nuclear Magnetic Resonance, sometimes called MRI or Magnetic Resonance Imaging) scans, and will probably need to examine radiographs of your baby before and after surgery. Any special scanning procedure that your baby requires will be explained to you by a doctor.

Radiographer This is a person trained to perform radiographs and ultrasound scans.

Social Worker Social workers in a neonatal setting are able to help families with any practical issues that arise during the hospitalisation period – such as travel expenses, accommodation and care of other children – as well as offering emotional support and advice on benefits and family entitlements which may be available. In some units it is the social worker who takes on the role of support person for the family.

A paediatric surgeon is a consultant who specialises in the childhood conditions which require operations, and will be the doctor who performs the surgery on your baby. If the condition was diagnosed before delivery, hopefully you will have been introduced and the condition and operation explained.

Speech Therapist

Physiotherapist

Other Medical Professionals

A person trained in the body's movement by natural forces, who may be required to help with your baby's breathing post-operatively, and also with general development later.

The professions listed above are those most often involved in neonatal intensive care, but there may be others who you may come into contact with. Do not be afraid to ask who they are and what they do if you are unsure; they are there for your benefit, so make use of them!

This person is trained to help with speech and language development problems. They are also often very useful with babies with feeding problems because they have expertise in dealing with such difficulties.

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The TOF Child

Parents' Worries JWL Puntis BM DM FRCP FRCPCH, Consultant Paediatric Gastroenterologist, The General Infirmary at Leeds, also Senior Lecturer in Paediatrics and Child Health, University of Leeds.

X-rays Children with OA/TOF often require repeated X-rays in the early weeks of life, both before and after surgery. Both parents and doctors worry about the risk of X-rays, particularly whether they will increase the chance of cancer developing in childhood or later life. However, these risks are extremely low. In a recent study of small babies in a neonatal unit,' many of whom had a considerable number of X-rays, the calculated dose of radiation received was reassuringly small. Even in the child who had the largest number of X-rays (far more than most TOFs need), the amount of radiation was estimated to be equivalent to three months of the natural background radiation to which we are all exposed in our daily life. The increased risk of cancer in this child was worked out as being 1 in 60,000. Despite this low risk, doctors are taught to carefully consider whether or not any X-ray examination is really necessary before making a request, and the smallest dose of radiation possible is used. Overall, the benefits of any Xrays in terns of treating the child far outweigh any potential radiation risks.

Once again it is important for doctors to be certain that a procedure requiring an anaesthetic is really necessary (usually there is little doubt about this) or that there is a bacterial infection demanding treatment. Children operated on for TOF will almost certainly be anaesthetised by a very experienced senior anaesthetist who has special expertise in dealing with young children. The most likely risk from antibiotics is a reaction to the drug, for example a rash, or diarrhoea. These usually resolve when the antibiotic is stopped and are rarely life threatening. Parents may worry that children will 'get used to' antibiotics, so that their effectiveness wanes over time. This is not the case, although very frequent (almost continuous) antibiotic treatment may encourage the development of germs in the body that become resistant to the effects of the antibiotic. This would be an unusual situation in the child with TOF who requires intermittent treatment, and in fact is rarely a problem in those children with other diseases such as cystic fibrosis who need intermittent courses of antibiotics for their whole life.

Antibiotics and Anaesthetics TOF children may also need a number of anaesthetics and frequent courses of antibiotics. The situation is very similar to that of X-rays in that there are risks involved, but they are very small and greatly outweighed by the risks of not having an anaesthetic or failing to treat an infection.

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References 1. Sutton P.M., Arthur R.J., Taylor C., Stringer M.D. (1998). Ionising radiation from diagnostic x rays in very low birthweight babies. Arch Dis Child: 78: F227-F229.


The TOF Child

Paediatric Resuscitation David A Needham GIFireE, REMT(p), DipAdEd, Medical Rescue, Nottingham

Every parent or carer of a child worries about the possibility of their child choking or being involved in some other mishap that stops them breathing. Anyone with a TOF child will almost certainly worry about this kind of thing even more. A respiratory arrest (inability to breathe) catches some TOF parents completely unawares because they have not been given the slightest hint from medical staff that this may occur. Medical staff sometimes withhold information from the parents of a sick child in the (often misguided) belief that the truth will terrify them. If however the risk of respiratory arrest is not explained, parents can then find themselves in a critical situation, the outcome of which is largely dependent upon them, for which they have not had the opportunity to confront in their minds – let alone train for. Some TOF children have what is known as a "floppy trachea" and a few may need to undergo corrective surgery for this problem. Whilst these children will have a slightly increased risk of choking, it does not automatically follow that they will definitely have a fatal choking episode. The number of children who die of asphyxia in the UK is very small. Respiratory arrest in children is usually recognised quickly and action is taken before it degenerates into cardiac arrest as well. Whilst most Accident and Emergency Department staff are unaware of the complexities of the TOF child's problems, and some may not even have heard of the condition, they are well trained to deal with the acute

crisis of a non-breathing child, whatever the cause may be. Rather than becoming continually fearful about their TOF child suffering respiratory arrest – even making themselves ill with worry – it is far mor e usef ul f or par ents t o channel energy into working out an action plan so that everyone knows what action to take if the occasion should arise. Having some notion of what to do makes the risk of respiratory arrest much less of a concern; parents cope much better once they are confident about what to do. Many parents have successfully resuscitated their child; some have done it more that once. Parents should realise that the outcome does not have to be bad; you can make a difference by preparing now in case the crisis occurs. Resuscitation is a skill and skills are best learned through doing. Most TOF parents leaving paediatric units will be taught how to resuscitate their baby using a mannikin. Failing this, the most appropriate way to prepare is by attending a training session in resuscitation (organisations and addresses can be found at the end of this chapter). Additionally, inside the front and back covers of this book you will find charts on resuscitation (one for infants, the other for children) which you can refer to, if the need should ever arise. A lot of misinformation about resuscitation is often revealed at resuscitation classes and this needs to be clarified. The following pages provide answers to some of the common questions that people ask (or sometimes are too self-conscious to ask).

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The TOF Child What should I do if my child is choking and coughing? The fact that your child is coughing is a good sign; it shows that they still have some control over their airway and they are trying to eject the foreign matter. In order to cough, the child has t o t a k e a i r i n t o t h e i r l u n g s a n d therefore they are still breathing. However, this threat to the airway may deteriorate to a complete obstruction and then cessation of breathing, so some supervision is required. Calmly encourage the child to cough, incline the child forward so that gravity is helping the object to fall out of the mouth. If this does not help, slap the child on the back between the shoulder blades with the heel of the hand to try to dislodge the foreign matter. If the foreign body still remains stuck, then you must resort to abdominal or chest thrusts to dislodge it. How much air should I blow into the child when I am performing mouth to mouth? This obviously depends on the size of the child or infant. As a general rule, the amount of air that you could hold in your mouth with your cheeks puffed out is about right for small infants. Larger children will obviously need more air to ventilate their lungs and it is important that you look at the child's chest when blowing into them. When the chest rises, you have given enough air. If you continue to force air in after the chest has f ull y risen then you may cause damage to the lungs, but what is more likely is that the air will follow the route of least resistance and end up in the child's stomach. This may cause vomiting, or more seriously it may fill the stomach so full of air that the lungs

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cannot expand properly; this would eventually lead to you not being able to blow any air into the child's lungs. Your lungs can easily expel 1.5 litres of air in one long breath. A child's lungs will only take 0.5 litres, or possibly less. Remember this and do not be over-zealous. I have heard about mouth to nose resuscitation; is this more effective than mouth to mouth? The short answer is no. Mouth to mouth and mouth to nose are equally efficient, but sometimes circumstances make it difficult for people to get an effective seal around the child or infant's mouth. In that case, do not hesitate to use mouth to nose. In the past, the teaching has been to carry out mouth to mouth-and-nose when resuscitating infants. Recent research shows that mothers do not have a sufficiently large mouth to form an adequate seal over both the mouth and nose of their infant. My advice to people is to ensure that a good seal is made to connect with the child or infant's airway, even if that means only doing mouth to nose. When I did a first aid course, we always checked for a pulse at the neck, and not the wrist. I have since then heard that you should check an infant's pulse on the upper arm. Which is correct? A pulse can indeed be obtained at all these sites, however the best to use varies with the individual situation and what you are trying to achieve. Checking for a pulse at the wrist is OK in some circumstances, but is never acceptable if someone has stopped breathing and you need to detect whether their heart has stopped.


The TOF Child In infants, the pulse in the neck is often difficult to locate due to their chubby necks. You may find it easier to detect a pulse on the inside of the upper arm, behind the biceps muscle.

What if I am not completely sure that my child has stopped breathing — can I do harm by beginning mouth to mouth?

NB When measuring the pulse rate in beats per minute, don't spend a whole minute doing it! Count the beats for 15 seconds; double this figure, then double it again (i.e. multiply it by four; doubling it twice is easier when under pressure!) to give the pulse rate for a minute. Practise this when your child is well and sleeping or sitting on your knee.

If it is such a fine judgement about whether a child is breathing or not, then start mouth to mouth without delay. In practical terms, you will not cause any harm. If the child makes respiratory efforts (i.e. tries to breathe) then you should try to synchronise your breaths with this respiratory effort.

What if I can detect a pulse, but it is very slow and weak, and my child is unconscious? Because the pulse is slow and weak, this means that the heartbeat is slow and weak. This in turn means that the blood supply to the brain will not be adequate – and in fact this is probably the reason that the child is unconscious. You must begin chest compressions on any infant or child who has a pulse rate of less than 60 per minute. This will increase the blood output from the heart and will therefore increase blood flow to the brain, which is critical to survival. What should I do if my child stops breathing, but still has a fast strong pulse? If you encounter these circumstances, then you have caught the problem at a very early stage, before respiratory arrest deteriorates to cardiac arrest. If you take the correct action promptly, the outcome of the episode is likely to be good. Gi ve mout h to mouth ( or mout h to nose) at a rate of 1 breath every 5 seconds, and continue to monitor the pulse. If the pulse stops or drops below 60 per minute, then commence chest compressions as well as mouth to mouth.

I have heard that if someone has not been breathing for 4 minutes, irreversible brain damage will o c c u r . B e a r i n g t h i s i n m i n d , should I stop mouth to mouth after four minutes if the person is not breathing for themselves? Absolutely not! You are providing the person with oxygen through your mouth to mouth ventilation, and you are circulating this oxygen round the body in the bloodstream by the chest compressions you are doing. You are the only thing that is keeping their brain alive. By doing mouth to mouth (and chest compressions when necessary) you are "buying time" for the ambulance crew to arrive and commence advanced emergency care techniques. Do not stop!

Resuscitation courses Medical Rescue Contact David Needham; address available from the TOFS office. St John Ambulance Contact the local division or the County Office. In Scotland, contact the Director General in Glasgow or your local Committee Secretary. British Red Cross Contact your local branch headquarters.

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The TOF Child

Glossary Vicki Martin BSc BVSc and Sue Goodley, Nottingham Neonatal Service. amniocentesis: a test whereby amnotic fluid is sampled by insertion of a needle. Tests on this fluid can give information about the baby's health. amniotic fluid: water around the baby in the womb. anastomosis: 'join' — as in oesophageal anastomosis, where the two ends of the oesophagus are stitched together in oesophageal atresia. antibiotic: medication used to treat bacterial infections. anus: external opening of the rectum (backpassage)... anal: relating to the anus. aortopexy: surgical procedure used in the management of severe tracheomalacia, in which the aorta (a major artery which lies in front of the trachea) is anchored to the sternum (breast bone). This has the effect of opening up the trachea. asthma: respiratory disease often associated with difficulty in breathing and a cough. atresia: term taken from ancient Greek, meaning 'no passage / no way through.' For example, in oesophageal atresia there is a break in the continuity of the oesophagus. bacteria: cellular agent causing disease... distinct from a virus. barium: is often used as a contrast agent in radiography: it shows up on radiographs (`Xrays')... a barium meal can be swallowed to show up the gastro-intestinal tract... a barium swallow shows up the oesophagus... a barium study is a sequence of radiographs taken over a period of time to show the passage of material through the gastro-intestinal tract. bowel: intestines... the small bowel is the small intestine (upper intestinal tract, that which follows after the stomach): the large bowel is the large intestine (lower intestinal tract, between the small intestine and the rectum). bronchi, bronchiole: the branching airways after the splitting of the trachea (bronchi then bronchioles). bronchoscopy: endoscopic examination of the airways. cardiac: related to the heart.

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CHARGE: acronym describing a group of anomalies which are sometimes associated with TOF/OA. The letters stand for: Coloboma (defects affecting the pupil of the eye), Heart disease, Atresia of the choanae (the passages at the back of the nose, Retarded growth, Genital hypoplasia (underdevelopment of the genital organs) and Ear anomalies/deafness. congenital: deformities or diseases which are either present at birth or, having been transmitted direct from parents to offspring, become obvious some time after birth. contra-indication: term which relates to a medicine or procedure... a contra-indication describes a situation in which that medicine or procedure should not be administered or carried out. For example, asthma is a contraindication for the drug ibuprofen. CPAP: Continuous Positive Airway Pressure —the application of a continuous positive pressure to the airways, used (for example) in the management of tracheomalacia to keep the airways open. dilatation: term used to describe a procedure undertaken to widen a narrowing (stricture). distal: the end furthest away (cf. proximal: the closest end). duodenum: the first part of the small intestine, just after the stomach. e l e c t r ol y t e s : m e d i c a l u s a g e o f t h i s t e r m relates to the salts found in body fluids, for example sodium, potassium, chloride, calcium etc... electrolyte imbalance: abnormality of the relative concentrations of body salts. endoscope: medical instrument with a tubelike structure, through which the inside of the body can be examined. Endoscopes can be rigid or flexible tubes incorporating either fibreoptic or video technologies: the image is seen either via an eyepiece or on a television screen. fistula: term from the Latin meaning `pipe...' an abnormal connection running either between two tubes or between a tube and a surface. In tracheo-oesophageal fistula it runs between the trachea and the oesophagus.


The TOF Child fluids: medical usage of this term relates to the administration of fluids into the blood stream, via an intravenous drip or pump. foreign body: medical usage of this term relates to the abnormal presence of a particle or object... an oesophageal foreign body is a swallowed item which is abnormally present in the oesophagus. gastric: relating to the stomach. gastrostomy: surgically-created connection between the inside of the stomach and the body wall. gastro-oesophageal reflux: the involuntary return of gastric (stomach) contents to the lower end of the oesophagus. gullet (see oesophagus). haematology and biochemistry: the study of the blood components... haematology relates to the cellular content, biochemistry to the chemical components. hypoplasia: decreased (less-than-normal) growth. immunity: resistance to disease... immune system: the body's armoury of defences producing resistance to disease. immunisation: the artificial creation of immunity against specific diseases. incision: surgical cut. inflammation: the body's reaction to injury (caused by e.g. trauma or disease). antiinfiammatories: drugs given to reduce inflammation. inpatient: patient receiving medical attention while resident in the hospital (cf. outpatient: patient seen during a short visit to hospital). kyphosis: abnormal curvature of the spine to give the appearance of a 'rounded back.' ligate: surgical term — to 'tie off' with a knot. medical: non-surgical management of disease (vs. surgical, involving physical intervention i.e. an operative procedure). mucus: moist fluid which is found (for example) in the normal airways where it helps to trap small particles e.g. dust. nebuliser: device which administers a drug to the airways. neonatal: newly born. oesophagus: gullet, the tube taking food from the mouth to the stomach. oesophageal incoordination: lack of coordination of the muscular activity of the oesophagus.

oesophageal substitution procedure: replacement of (a part of) the oesophagus with another part of the gastro-intestinal tract. oesophagostomy: surgical creation of a connection between the oesophagus and the body wall (skin)... cervical oesophagostomy: connection of the oesophagus to the neck. oral: relating to the mouth. outpatient: patient seen during a short visit to the hospital (cf. inpatient: patient receiving medical attention whilst resident in hospital). paediatric: relating to children. paediatrician: doctor who has specialised in the treatment of childhood disorders. peristalsis: wave of muscular activity, in e.g. the oesophagus or intestine, which transports the contents along the gut. pH: acidity... pH monitoring: the monitoring of pH (performed in the diagnosis of gastrooesophageal reflux, in which case the monitor is positioned at the base of the oesophagus, j u s t before it enters the stomach). pneumonia: inflammation of the lungs. aspiration pneumonia is caused by the entrance of fluids into the respiratory tract. pollicization: the surgical reconstruction of thumb by transferring the index finger. polyhydramnios: condition in which an abnormally large amount of fluid is present in the womb: often associated with TOF/OA in the baby. Also known as simply 'hydramnios.' prenatal: before birth. primary health care team: the team of medical professionals who are involved in local health care... includes (e.g.) the general practitioner, practice nurse, health visitor etc. progeny: offspring (sons and daughters). proximal: the closest end (cf. distal: the end furthest away). radiograph: an X-ray image... radiography: the taking of X-rays. radiology: the study/interpretation of radiographs. radial dysplasia/aplasia: abnormality of development of the radius, a bone in the forearm: this is a condition affecting some VACTERL children. rectum: backpassage, the last part of the gastro-intestinal tract. reflux (see gastro-oesophageal reflux). renal: relating to the kidneys.

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The TOF Child respiration: breathing... respirator: a machine which 'breathes for' the patient... respiratory distress syndrome: complex condition affecting newborn babies, in which respiration does not occur normally. resuscitation: life-saving procedure(s) involving bringing an unconscious person back to consciousness by stimulation of the respiratory and/or circulatory systems. scoliosis: abnormal curvature of the spine in a sideto-side direction. sham feeding: procedure carried out with babies who have not received an anastomosis (join-up operation) on their oesophagus, but have received a cervical oesophagostomy and gastrostomy. Food is given by mouth, but exits at the neck through an oesophagostomy (a surgically created opening of the upper oesophagus at the neck). Simultaneously, food is given straight into the stomach via the gastrostomy tube (a surgically-created opening of the stomach at the body wall). In this way the child learns to eat and swallow, and to associate this with the feeling of `being full,' in preparation for later li fe when their mouth has been surgically reconnected to their stomach and normal feeding can begin. stricture: narrowing... most often - but not always — referring to a narrowing of the oesophagus at the anastomosis (join-up) site. Can also be congenital (present at birth) or subsequent to gastro-oesophageal reflux. sutures: surgical stitches. symptom: the problem reported by the patient. TOF cough: characteristic 'barking' cough often exhibited by TOF children, caused by a degree of tracheomalacia. trachea: windpipe... the main airway from the mouth/nose to the branching bronchi and bronchioles which lead into the lungs. tracheomalacia: 'floppiness' of the trachea. tracheopexy: surgical procedure used in the management of tracheomalacia, in which the trachea is anchored to the sternum (breast bone) in order to open up the airway. tracheostomy: the surgical creation of a connection between the trachea and the skin at the neck, and the insertion of a tube into the trachea to preserve a clear airway. Used in the management of severe tracheomalacia.

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transanastomotic tube: a tube which is placed in the oesophagus and runs across the site of an anastomosis (join-up) into the stomach. This enables the baby to be fed milk while the operation site heals. ulna: bone in the forearm: the upper end of the ulna is the so-called 'funny bone.' ultrasonography: diagnostic imaging procedure in which an ultrasound scan is performed which produces 'real-time' images of the internal body structures using sound waves. VATER: acronym for a group of disorders often occurring together. The letters stand for Vertebral (spine), Anal (backpassage), Tracheal, Esophageal (from the American spelling; the UK spelling is `oesophageal'), Renal (related to the kidney) and Radial (the radius bone in the forearm). VACTERL: more correct/up-to-date acronym, in place of VATER (above): the extra letters in VACTERL stand for Cardiac (affecting the heart) and Limb (since there a r e o the r l i mb abnor ma li ti e s oth er tha n those involving only the radius). ventricular septal defect (VSD): defect in the wall dividing the left and right ventricles (the lower chambers in the heart) meaning that oxygenated and deoxygenated blood mixes and the transportation of oxygen from the lungs to the tissues is inefficient. The circulation in the lungs is also subject to abnormally high pressures, which can be damaging. ventilation: medical usage generally refers to mechanical ventilation, in which a machine `breathes' for the patient (the machine is called a ventilator, which is the same as a respirator). vertebra: each of the bones which makes up the spine is called a vertebra. virus: infective agent causing disease... distinct from a bacteria and not susceptible to antibiotics. vital functions: term often used to refer to the basic bodily functions monitored during patient care, e.g. breathing, circulation and temperature. vomiting: 'being sick.' VSD (see ventricular septal defect). weaning: introducing solid food as well as milk into the baby's diet. windpipe (see trachea). X-ray (see radiograph).


The TOF Child

Index A acid lowering drugs 68 advanced neonatal nurse practitioner 118 Aerobec 79 Aerolin 79 alpha feto protein 10 amniocentesis 10 anaesthetics 120 anastomosis 22 anlage 107 anorectal atresia 100 antibiotics 77, 120 for renal anomalies 101 antireflux medicines 68 antireflux surgery 69 aortopexy 77 aspiration pneumonia 32 asthma 78 atresia. See oesophageal atresia Atrovent 79

B Barrett's oesophagitis 29 Beck, JM 37 Becotide 79 Bland, David 5 b l u e d o ' s 4 0 bonding 16 Bricanyl 79 bronchodilators 79 Brown, Leslie 60, 118 Buckler Tanner chart 82 Buick, RG 30 Butcher, Eleanor 32

C cardiac abnormalities 100 Carobel 68 centralisation 109 cervical oesophagostomy 26 chaplain 117 CHARGE 14 chest drain 24, 33 chest infections 47, 77, 95 Chetcuti, PAJ 82, 95 Cimetidine 68 Cisapride 68 Clamp, Janita 6 ClampGray, Freddie 59 cleft lip 98 club hand 107 colon interposition 27 colostomy 100 community midwife 116

Dickson, JAS 7 dietician 117 dilatation 63, 95 distraction lengthening 109 D o b b s , B e n 2 0 Dodd, Emma Louise 29 domperidone 68 Downs syndrome 49 dumping 28, 70

E ear abnormalities 98 education 95 Edward's syndrome 49, 99 endoscopy for diagnosis of OA 12 for diagnosis of reflux 67 for diagnosis of stricture 62 for diagnosis of TOF 12 for stricture 63 for tracheomalacia 76 exercise 95

F feed thickeners 68 feeding 52-57 breast feeding 52 oral feeding 39 postoperatively 33 problems 54 reluctance to feed 54 sham feeding 44 solid foods 52 troublesome foods 55 tube feeding 40 weaning 52 Firth, Helen 49 fistula. See tracheooesophageal fistula

friends 91

G gas bloat 70 ga s tr ic tr a nsp o s it io n 2 8 gastric tube oesophagoplasty 28 gastro-oesophageal reflux 35, 40, 44, 55, 64, 67, 72 diagnosis 66-67 surgery 69 symptoms 65 gastrostomy 24, 41, 69 care of 43 for reflux 69 Gaviscon 68 general practitioner 116 genetic counselling 49 geneticist 117

genital anomalies 98 Gibson, Thomas 7 Goodley, Sue 6, 116 Gordon, Ben 104 grandparents 87 growth 82-85 growth hormone 83

H haematemesis 65 health visitor 116 Holt-Oram anomaly 113 horseshoe kidney 101 hospital liaison visitor 39, 85 hospital midwife 117 hydramnios. See polyhydramnios hypoplastic kidneys 101 I imperforate anus 100 Intal 79 Intensive Care Unit 30 Intensive Therapy Unit 30 ipratropium 79 J jaundice 35 jejunostomy 29, 42

K Kapila, L 98 Kaufman syndrome 98 Kay, Simon PJ 106 Kluth, Dietrich 8 kyphosis 99

L Lander, AD 30 laryngeal cleft 8 Lawton, Sandra Kay 97 Lee, R 98 liaison health visitor 117 limb abnormalities 103 Ludman, Lorraine 15

M MacFadyen, Una M 71 Martin, Vicki 4 Mayo, Jacob 31, 35 medications 46 MedicAlert 6 Micolaud, Christine 97 milk scans 67 monitoring 30 motility drugs 68 Motilium 68

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The TOF Child N nasogastric tube 41 care of 42 Needham, David A 121 neonatal nurse 117 neonatologist 118 Nestargel 68 Nissen fundoplication 69 0 oesophageal atresia 8 conditions occurring with 14 diagnosis 12 long-gap 24 pre-natal diagnosis 10, 18 with lower pouch fistula 8 with upper pouch fistula 8 without fistula 8 oesophageal incoordination 55 oesophageal obstruction 55, 62 oesophagostomy. See cervical oesophagostomy ophthalmologist 119 P paediatric surgeon 119 paediatrician 118 Palmer, Craig 80 parents 15, 86 Parkinson, Glenys 86 pelvic kidney 101 pH Study 66 physiotherapist 119 physiotherapy 47 for radial dysplasia 108 PIAVA 98 Pierce, Anthony 40 PIV 98 play therapy 89 pollicisation 113 polycystic kidney 101 polyhydramnios 10, 51 Pouncey, Benjamin 5 practice nurse 117 Prepulsid 68 preventers 79 Pulmicort 79 Puntis, JWL 52, 120 R radial dysplasias 106 the hand anomaly 111-112 treatment of the elbow 108 treatment of the forearm 107-111 treatment of the hand 112 treatment of the wrist 108-111

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radialisation 110 radiographer 119 radiography for OA 12 for reflux 66 for stricture 62, 64 for tracheomalacia 76 radiologist 119 ranitidine 68 registrar 119 relievers 79 r e n a l anomalies 101 respiratory arrest 121 respiratory distress syndrome 21 respiratory function tests 76 resuscitation 38, 121 S salmeterol 79 Sancto, Hannah and Rebecca 51 Say-Gerald Syndrome 98 school 92, 96 scoliosis 99 senior house officer 119 Serevent 79 Seymour, Penny 37 Shore, Celia 52 siblings 89 single umbilical artery 98 social worker 119 sodium cromoglycate 79 Special Care Baby Unit 30 speech therapist 41, 119 S p e n d e r , K r i s 3 4 Spitz, Prof L 21, 26 Sprigg, Alan 11 stricture 55, 61 anastomotic 61 and reflux 61 congenital 61 diagnosis 62 surgery 64 symptoms of 61 treatment 63-64 Stringer, Mark D 61 surgery for TOF/OA 21-25 discharge from hospital 37 follow up after 48 leaks after 34 long term outcome 95-96 oesophageal substitution procedures 26 post-operative care 30-35 preoperative management 21 primary anastomosis 22 surgical outreach nurse 39

T Tagamet 68 Thal fundoplication 69 theophylline 79 Thick and Easy 68 Thixo-D 68 thu mb ab no rmali ties 9 8 TOF cough 45, 73, 95 total parenteral nutrition 33 tracheo-oesophageal fistula 8 conditions occurring with 14 diagnosis 12 double fistula 8 H fistula 8 without atresia 8 tracheomalacia 34, 45, 72 tests for 76 treatment 77 tracheopexy 77 tracheostomy 77 transanastomotic tube 24, 31 transfer to another hospital 14, 15, 21 Treacher-Collins syndrome 49 Trump, Dorothy 49 U Uniphyllin 79 V vaccinations 37 VACTERL 98-104 antenatal diagnosis 99 VATER 98 ventilator 32 Ventolin 79 Ventricular septal defect (VSD) 100 vertebral abnormalities 99 vomiting 65, 95 w Wharfe, James 57, 85 wheeze 78, 95 Wood, Andrew 5 X X-rays 120 z Zantac 68


The TOF Child